Role of carnitine in disease

Flanagan, Judith L; Simmons, Peter A; Vehige, Joseph; Willcox, Mark DP; Garrett, Qian

doi:10.1186/1743-7075-7-30

Cited by 613Metabolism and Genetic DisordersClinical Nutrition and GastroenterologyMuscle metabolism and nutrition

Role of carnitine in disease

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Flanagan, Judith L, Simmons, Peter A, Vehige, Joseph, Willcox, Mark DP, Garrett, Qian·2010·Nutrition & Metabolism

DOI 10.1186/1743-7075-7-30PMC PMC2861661

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Abstract

Carnitine is a conditionally essential nutrient that plays a vital role in energy production and fatty acid metabolism. Vegetarians possess a greater bioavailability than meat eaters. Distinct deficiencies arise either from genetic mutation of carnitine transporters or in association with other disorders such as liver or kidney disease. Carnitine deficiency occurs in aberrations of carnitine regulation in disorders such as diabetes, sepsis, cardiomyopathy, malnutrition, cirrhosis, endocrine disorders and with aging. Nutritional supplementation of L-carnitine, the biologically active form of carnitine, is ameliorative for uremic patients, and can improve nerve conduction, neuropathic pain and immune function in diabetes patients while it is life-saving for patients suffering primary carnitine deficiency. Clinical application of carnitine holds much promise in a range of neural disorders such as Alzheimer's disease, hepatic encephalopathy and other painful neuropathies. Topical application in dry eye offers osmoprotection and modulates immune and inflammatory responses. Carnitine has been recognized as a nutritional supplement in cardiovascular disease and there is increasing evidence that carnitine supplementation may be beneficial in treating obesity, improving glucose intolerance and total energy expenditure.

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L-carnitine bioavailability is higher in vegetarians than in carnivores, so less carnitine reaches lower-intestine bacteria in vegetarians.
“The bioavailability of L-carnitine in vegetarians (who are adapted to low-carnitine diets) was higher than in carnivores (42); therefore, less carnitine reaches the bacteria of the lower intestine in vegetarians.”
From Trimethylamine N -Oxide, the Microbiome, and Heart and Kidney Disease (2017) · cites this paper as [42]
Carnitine can be synthesized endogenously from lysine and methionine in kidney, liver, and brain.
“Carnitine also can be synthesized endogenously from two essential amino acids, lysine and methionine (63).This occurs in kidney, liver, and brain (42).”
From Trimethylamine N -Oxide, the Microbiome, and Heart and Kidney Disease (2017) · cites this paper as [42]
Animal products such as meat, fish, poultry, and milk are highest in carnitine, while plants contain little carnitine.
“Animal products like meat, fish, poultry, and milk (whey fraction) are highest in carnitine, whereas plants contain little carnitine (42).”
From Trimethylamine N -Oxide, the Microbiome, and Heart and Kidney Disease (2017) · cites this paper as [42]

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Background
C75, a Fatty Acid Synthase Inhibitor, Modulates AMP-activated Protein Kinase to Alter Neuronal Energy Metabolism
Landree, L. E., Hanlon, A. L., Strong, D. W., Rumbaugh, G., Miller, I. M., Thupari, J. N., Connolly, E. C., Huganir, R. L., Richardson, C. A., Witters, L. A., et al. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m310991200 · Cited by 131 · cited 1× · see citing works
“There is some debate in the literature regarding whether satiety depends on the cytosolic concentration of long-chain fatty acids, with the suggestion that an increased concentration correlates with satiety and decreased feeding and body weight [ 77-80 ].”
Background
Carnitine and its derivatives in cardiovascular disease
Arsenian, M. A. (1997) · Progress in Cardiovascular Diseases · doi:10.1016/s0033-0620(97)80037-0 · Cited by 123 · cited 1× · see citing works
“L-carnitine has been shown to have favorable effects in patients with severe cardiovascular disorders, such as coronary heart disease, chronic heart failure and peripheral vascular disease [123-125].”
Background
Diagnosis and management of glutaric aciduria type I
Barić, I., Zschocke, J., Christensen, E., Durán, M., Goodman, S. I., Leonard, J. V., Müller, E., Morton, D. H., Superti‐Furga, A., & Hoffmann, G. F. (1998) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1005390105171 · Cited by 122 · cited 1× · see citing works
“GCDH can be controlled nutritionally and supplementation includes L-carnitine to avoid secondary carnitine depletion [84-87].”
Background
Human Tear Film Electrolyte Concentrations in Health and Dry-Eye Disease
Gilbard, J. P. (1994) · International Ophthalmology Clinics · doi:10.1097/00004397-199403410-00005 · Cited by 122 · cited 1× · see citing works
“Tears of patients with dry eye show significantly increased osmolarity, with a mean value of 343 mOsm compared with 302 mOsm in healthy controls [162].”
Background
Pathomechanisms of neurodegeneration in glutaryl‐CoA dehydrogenase deficiency
Kölker, S., Koeller, D. M., Okun, J. G., & Hoffmann, G. F. (2003) · Annals of Neurology · doi:10.1002/ana.10784 · Cited by 121 · cited 1× · see citing works
“Secondary carnitine depletion due to increased formation and urinary excretion of glutarylcarnitine is suggested to play an important role in the neuropathogenesis of GCDH deficiency, inducing excitotoxic neuronal damage and mitochondrial dysfunction [83].”
Background
Isolation and characterization of cDNA and genomic clones encoding human muscle type carnitine palmitoyltransferase I
Yamazaki, N., Shinohara, Y., Shima, A., Yamanaka, Y., & Terada, H. (1996) · Biochimica et Biophysica Acta (BBA) - Gene Structure and Expression · doi:10.1016/0167-4781(96)00069-3 · Cited by 119 · cited 1× · see citing works
“The nucleotide sequences of cDNA and genomic DNA encoding human CPTI have been characterized [45,46].”
Method
Inhibitory Effects of Alcohol on Intestinal Amino Acid Transport in vivo and in vitro
Israel, Y., Salazar, I., & Rosenmann, E. (1968) · Journal of Nutrition · doi:10.1093/jn/96.4.499 · Cited by 117 · cited 1× · see citing works
“It is known that dietary absorption of amino acids is impaired by ethanol so this could also contribute to carnitine deficiency overall [73].”
Background
Propionyl-l-carnitine prevents the progression of cisplatin-induced cardiomyopathy in a carnitine-depleted rat model
Al‐Majed, A. A., Sayed‐Ahmed, M. M., Al‐Yahya, A. A., Aleisa, A. M., Al-Rejaie, S. S., & Al‐Shabanah, O. A. (2006) · Pharmacological Research · doi:10.1016/j.phrs.2005.12.005 · Cited by 115 · cited 1× · see citing works
“Al-Majed and colleagues [ 140 ] found that carnitine deficiency and oxidative stress are risk factors during development of cisplatin (CDDP)-induced cardiomyopathy and that carnitine supplementation, using propionyll-carnitine, prevents the progression of CDDP-induced”
Background
Carnitine Palmitoyltransferase II Deficiency: A Clinical, Biochemical, and Molecular Review
Sigauke, E., Rakheja, D., Kitson, K., & Bennett, M. J. (2003) · Laboratory Investigation · doi:10.1097/01.lab.0000098428.51765.83 · Cited by 111 · cited 2× · see citing works
“The perinatal disease is the most severe form and is invariably fatal [34].”
Background
Plasma Carnitine Deficiency
Winter, S. C. (1987) · American journal of diseases of children · doi:10.1001/archpedi.1987.04460060076039 · Cited by 106 · cited 1× · see citing works
“Finally, incremental growth was seen in 22 of 33 carnitine-administered patients who presented with failure to thrive [110] and this was attributed to the role of carnitine as a muscle growth factor.”
Background
Acetyl-l-Carnitine Treatment in Minimal Hepatic Encephalopathy
Malaguarnera, M., Gargante, M. P., Cristaldi, E., Vacante, M., Risino, C., Cammalleri, L., Pennisi, G., & Rampello, L. (2008) · Digestive Diseases and Sciences · doi:10.1007/s10620-008-0238-6 · Cited by 105 · cited 2× · see citing works
“Minimal hepatic encephalopathy (MHE) is a serious and common complication that occurs in the majority of cirrhotic patients [ 59 ].”
Background
Effects of L-carnitine in patients with hepatic encephalopathy
Malaguarnera, M. (2005) · World Journal of Gastroenterology · doi:10.3748/wjg.v11.i45.7197 · Cited by 103 · cited 2× · see citing works
“One suggested mechanism of carnitine action is its reduction of serum ammonia levels leading to improved psychometric measures [63-65].”
BackgroundResult
Current status of genetics research in inflammatory bowel disease
Vermeire, S., & Rutgeerts, P. (2005) · Genes and Immunity · doi:10.1038/sj.gene.6364257 · Cited by 102 · cited 3× · see citing works
“Carnitine transporter mutations in Crohn's disease consists of missense mutation(s) in the gene coding plasma membrane transporter OCTN1 (SLC22A4) and/ or mutation(s) in the promoter of the gene encoding OCTN2 (SLC22A5) [14,35].”
Background
Carnitine deficiency syndromes
Breningstall, G. N. (1990) · Pediatric Neurology · doi:10.1016/0887-8994(90)90037-2 · Cited by 101 · cited 1× · see citing works
“Uptake in skeletal and cardiac muscle is a saturable active transport process against a concentration gradient [ 16 ].”
Background
Ophthalmologic findings in long-chain 3-hydroxyacyl-Coa dehydrogenase deficiency caused by the G1528C mutation
Tyni, T. A., Kivelä, T., Lappi, M., Summanen, P. A., Nikoskelainen, E. K., & Pihko, H. S. (1998) · Ophthalmology · doi:10.1016/s0161-6420(98)95019-9 · Cited by 101 · cited 1× · see citing works
“Chorioretinopathy emerges during early childhood as granular pigmentation of the central fundus with or without pigment clumping which may progress to chorioretinal atrophy, high myopia, posterior staphyloma and low vision [165].”
Background
Age-dependent decrease of carnitine content in muscle of mice and humans
Costell, M., O’Connor, J., & Grisolı́a, S. (1989) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(89)91360-0 · Cited by 100 · cited 1× · see citing works
“L-carnitine levels in tissues have been found to decline with age [142].”
Background
Mitochondrial dysfunction and neuromuscular disease
Nardin, R. A., & Johns, D. R. (2001) · Muscle & Nerve · doi:10.1002/1097-4598(200102)24:2 · Cited by 100 · see citing works
Ameliorating effects of carnitine and its precursors on alcohol-induced fatty liver
Sachan, D. S., Rhew, T. H., & Ruark, R. A. (1984) · American Journal of Clinical Nutrition · doi:10.1093/ajcn/39.5.738 · Cited by 99 · cited 4× · see citing works
“Supplementation of the diet with lysine can restore carnitine levels, however, there appeared to be impairment of carnitine biosynthesis in ethanol-compromised livers in the rat study [71].”
BackgroundInfluential
Valproate (VPA) Metabolites in Various Clinical Conditions of Probable VPA‐Associated Hepatotoxicity
Siemes, H., Nau, H., Schultze, K. D., Wittfoht, W., Drews, E., Penzien, J. M., & Seidel, U. (1993) · Epilepsia · doi:10.1111/j.1528-1157.1993.tb02419.x · Cited by 98 · cited 1× · see citing works
“Benefits of oral L-carnitine in relation to VPA-associated deficiency and related adverse effects have been reported [137-139].”
Background
Current concepts in carnitine research
Carter, A. L. (1992) · Cited by 97 · see citing works
L‐Carnitine in Dialysis Patients
Ahmad, S. (2001) · Seminars in Dialysis · doi:10.1046/j.1525-139x.2001.00055.x · Cited by 96 · cited 5× · see citing works
“Impairment of excretion of AC occurs with deteriorating renal function leading to decreased carnitine clearance and resulting in elevated plasma levels of carnitine [13].”
BackgroundInfluential
Carnitine Abnormalities in Patients with Renal Insufficiency
Wanner, C., & Hörl, W. H. (2008) · The Nephron journals/Nephron journals · doi:10.1159/000185137 · Cited by 94 · see citing works
The Role of Inflammatory Markers in the Cardioprotective Effect of L-Carnitine in L-NAME-Induced Hypertension
Miguel‐Carrasco, J. L., Mate, A., Monserrat-García, M. T., Arias, J. L., Aramburu, Ó., & Vázquez, C. M. (2008) · American Journal of Hypertension · doi:10.1038/ajh.2008.271 · Cited by 90 · cited 1× · see citing works
“[121] demonstrated in a rat model that chronic administration of LC reduces blood pressure and attenuates the inflammatory process associated with arterial hypertension.”
Background
Primary and secondary alterations of neonatal carnitine metabolism
Scaglia, F., & Longo, N. (1999) · Seminars in Perinatology · doi:10.1016/s0146-0005(99)80047-0 · Cited by 89 · cited 1× · see citing works
“Secondary carnitine deficiency can be caused by increased losses, pharmacological therapy, a number of inherited metabolic disorders [38], poor diet or malabsorption of carnitine, from increased renal tubular loss of free carnitine (Fanconi syndrome), haemodialysis, peritoneal dialysis, or the increased excretion of a…”
Background
Different sensitivity of rabbit heart and skeletal muscle to endotoxin‐induced impairment of mitochondrial function
Trumbeckaitė, S., Opalka, J. R., Neuhof, C., Zierz, S., & Gellerich, F. N. (2001) · European Journal of Biochemistry · doi:10.1046/j.1432-1327.2001.02012.x · Cited by 89 · cited 1× · see citing works
“It has been further suggested that carnitine deficiency might negatively impact cardiac function which might in turn, further contribute to the outcome of patients suffering sepsis [97-99].”
Background
Mutations in Novel Organic Cation Transporter (OCTN2), an Organic Cation/Carnitine Transporter, with Differential Effects on the Organic Cation Transport Function and the Carnitine Transport Function
Seth, P., Wu, X., Huang, W. Y., Leibach, F. H., & Ganapathy, V. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.47.33388 · Cited by 85 · cited 1× · see citing works
“Several mutations have been described [21,22,25,26,29,31-33].”
Carnitine and hemodialysis
Bellinghieri, G., Santoro, D., Calvani, M., Mallamace, A., & Savica, V. (2003) · American Journal of Kidney Diseases · doi:10.1053/ajkd.2003.50099 · Cited by 85 · cited 1× · see citing works
“Plasma concentration of free carnitine is in dynamic balance with acylcarnitines with the acyl to fee carnitine ration of ≤ 0.4 being considered normal [ 11 ].”
MethodInfluential
Dietary management of long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency (LCHADD). A case report and survey
Gillingham, M. B., van Calcar, S., Ney, D. M., Wolff, J. A., & Harding, C. (1999) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1005437616934 · Cited by 84 · cited 1× · see citing works
“However the dietary impact is controversial [166].”
Primary carnitine deficiency.
Scholte, H. R., Pereira, R. R., Jonge, P. C. D., Luyt‐Houwen, I. E. M., Hedwig, M., Verduin, M., & Ross, J. D. M. (1990) · PubMed · Cited by 81 · see citing works
Carnitines and Its Congeners: A Metabolic Pathway to the Regulation of Immune Response and Inflammation
Famularo, G., Simone, C. D., Trinchieri, V., & Mosca, L. N. (2004) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1320.012 · Cited by 78 · cited 4× · see citing works
“There has even been suggestion that maintenance of normal carnitine levels might inhibit muscle wasting, hepatic lipogenesis, hypertriglycerdemia and decreased fatty acid oxidation that are seen in sepsis [ 95 ].”
BackgroundInfluential
Carnitine: An overview of its role in preventive medicine
Kendler, B. S. (1986) · Preventive Medicine · doi:10.1016/0091-7435(86)90005-8 · Cited by 74 · cited 9× · see citing works
“Ascorbic acid, ferrous iron, pyroxidine and niacin are also necessary cofactors [1] and deficiencies of any of these can lead to carnitine deficiency.”
BackgroundInfluential
Clinical and biochemical features of fatty acid oxidation disorders
Rinaldo, P., Raymond, K., Al‐Odaib, A., & Bennett, M. J. (1998) · Current Opinion in Pediatrics · doi:10.1097/00008480-199810060-00014 · Cited by 73 · cited 2× · see citing works
“Patients with secondary carnitine deficiency accumulate organic acids which causes enhanced urinary excretion of carnitine in the form of acyl-carnitines [36,37].”
Background
Fatty acid oxidation in bone tissue and bone cells in culture. Characterization and hormonal influences
Adamek, G., Felix, R., Guenther, H. L., & Fleisch, H. A. (1987) · Biochemical Journal · doi:10.1042/bj2480129 · Cited by 72 · cited 1× · see citing works
“It has been shown that cells of the osteoblastic lineage generate 40-80% of their energy demands through fatty acid oxidation [153].”
Background
History of L-carnitine: Implications for renal disease
Matera, M., Bellinghieri, G., Costantino, G., Santoro, D., Calvani, M., & Savica, V. (2003) · Journal of Renal Nutrition · doi:10.1053/jren.2003.50010 · Cited by 71 · cited 2× · see citing works
“L-Carnitine supplementation an lead to improvements in several complications seen in uremic patients, including cardiac complications, impaired exercise and functional capacities, muscle symptoms, increased symptomatic intradialytic hypotension, and erythropoietin-resistant anemia through normalizing the reduced carni…”
Background
Transport of butyryl- l -carnitine, a potential prodrug, via the carnitine transporter OCTN2 and the amino acid transporter ATB0,+
Srinivas, S. R., Prasad, P. D., Umapathy, N. S., Ganapathy, V., & Shekhawat, P. (2007) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00233.2007 · Cited by 69 · cited 4× · see citing works
“Carnitine transporter mutations in Crohn's disease consists of missense mutation(s) in the gene coding plasma membrane transporter OCTN1 (SLC22A4) and/ or mutation(s) in the promoter of the gene encoding OCTN2 (SLC22A5) [14,35].”
BackgroundInfluential
Carnitine membrane transporter deficiency: a long-term follow up and OCTN2 mutation in the first documented case of primary carnitine deficiency
Cederbaum, S. D., Koo-McCoy, S., Tein, I., Hsu, B. Y. L., Ganguly, A. A., Vilain, E. J. N., Dipple, K. M., Cvitanović-Šojat, L., & Stanley, C. A. (2002) · Molecular Genetics and Metabolism · doi:10.1016/s1096-7192(02)00169-5 · Cited by 68 · cited 1× · see citing works
“Due to defective renal absorption (free) carnitine is excreted in the urine of patients with primary deficiency and can result in tissue carnitine levels dropping to below 10% of normal [14,22,24-26].”
Background
Carnitine Transporter OCTN2 Mutations in Systemic Primary Carnitine Deficiency: A Novel Arg169Gln Mutation and a Recurrent Arg282ter Mutation Associated with an Unconventional Splicing Abnormality
Burwinkel, B., Kreuder, J. G., Schweitzer, S., Vorgerd, M., Gempel, K. E., Gerbitz, K.-D., & Kilimann, M. W. (1999) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1999.1060 · Cited by 68 · cited 2× · see citing works
“Several mutations have been described [21,22,25,26,29,31-33].”
Background
Mitochondrial nutrients improve immune dysfunction in the type 2 diabetic Goto‐Kakizaki rats
Hao, J., Shen, W., Tian, C., Liu, Z., Ren, J., Luo, C. Y., Long, J., Sharman, E. H., & Liu, J. (2008) · Journal of Cellular and Molecular Medicine · doi:10.1111/j.1582-4934.2008.00342.x · Cited by 67 · cited 2× · see citing works
“Administration of a combination of mitochondrial targeting nutrients, including carnitine, suggested carnitine may be effective in improving immune function in type 2 diabetes through enhancement of mitochondrial function, decreased oxidative damage, and delayed cell death in the immune organs and blood [81].”
Background
Protective Effect of L–Carnitine in Ammonia–Precipitated Encephalopathy in the Portacaval Shunted Rat
Therrien, G., Rose, C. F., Butterworth, J., & Butterworth, R. F. (1997) · Hepatology · doi:10.1002/hep.510250310 · Cited by 66 · cited 2× · see citing works
“There is a strong correlation between hepatic encephalopathy and abnormal ammonia handling, and ALC has been shown to induce ureagenesis leading to decreased blood and brain ammonia levels [ 60 ].”
BackgroundResult
VALPROIC ACID ASSOCIATED ABNORMALITIES OF CARNITINE METABOLISM
Murphy, J., Marquardt, K. M., & Shug, A. (1985) · The Lancet · doi:10.1016/s0140-6736(85)91481-3 · Cited by 64 · cited 8× · see citing works
“Siemes H, Nau H, Schultze K, Wittfoht W, Drews E, Penzien J, Seidel U: Valproate (VPA) metabolites in various clinical conditions of probable VPA-associated hepatotoxicity.”
BackgroundInfluential
A randomised, double-blind, placebo-controlled trial of L-carnitine in suspected acute myocardial infarction
Singh, R. B., Niaz, M. A., Agarwal, P., Beegum, R., Rastogi, S. S., & Sachan, D. S. (1996) · Postgraduate Medical Journal · doi:10.1136/pgmj.72.843.45 · Cited by 63 · cited 2× · see citing works
“Xue and colleagues suggest that the beneficial effects of L-carnitine in cardiovascular disease are due to the resumption of normal oxidative metabolism and restoration of myocardial energy reserves [ 128 ,129].”
Background
Carnitine Replacement in End‐Stage Renal Disease and Hemodialysis
Calvani, M., Benatti, P., Mancinelli, A., D'Iddio, S., Giordano, V., Koverech, A., Amato, A., & Brass, E. P. (2004) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1320.005 · Cited by 63 · see citing works
PPARα agonists up-regulate organic cation transporters in rat liver cells
Luci, S., Geißler, S., König, B., Koch, A. H., Stangl, G. I., Hirche, F., & Eder, K. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.09.099 · Cited by 62 · cited 3× · see citing works
“Experiments with fasted and calorie-restricted rats showed increased mRNA concentrations of acyl-CoA and CPT-1 in the liver, heart and kidneys compared to control animals due to upregulation of PPARα [111].”
Background
Levels of carnitines in brain and other tissues of rats of different ages: Effect of acetyl-L-carnitine administration
Maccari, F., Arseni, A., Chiodi, P., Ramacci, M. T., & Angelucci, L. (1990) · Experimental Gerontology · doi:10.1016/0531-5565(90)90043-2 · Cited by 62 · cited 1× · see citing works
“Carnitine levels decrease with age [151].”
C-peptide and diabetic neuropathy
Sima, A. (2003) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.12.9.1471 · Cited by 61 · cited 1× · see citing works
“As the disease progresses however it becomes increasingly nonresponsive to therapeutic interventions [92,93].”
Background
Plasma carnitine levels in children with protein-calorie malnutrition before and after rehabilitation
Khan, L., & Bamji, M. S. (1977) · Clinica Chimica Acta · doi:10.1016/0009-8981(77)90513-7 · Cited by 61 · cited 1× · see citing works
“Malnutrition, fasting and vegetarianism Reduced plasma carnitine levels have been noted in malnourished children [ 106 ] and adults [107].”
Background
Prevention of cerebral palsy in glutaric aciduria type 1 by dietary management
Monavari, A. A. (2000) · Archives of Disease in Childhood · doi:10.1136/adc.82.1.67 · Cited by 61 · cited 1× · see citing works
“GCDH can be controlled nutritionally and supplementation includes L-carnitine to avoid secondary carnitine depletion [84-87].”
Background
Treatment of carnitine deficiency
Winter, S. C. (2003) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1024433100257 · Cited by 60 · cited 1× · see citing works
“Secondary carnitine deficiency can be caused by increased losses, pharmacological therapy, a number of inherited metabolic disorders [38], poor diet or malabsorption of carnitine, from increased renal tubular loss of free carnitine (Fanconi syndrome), haemodialysis, peritoneal dialysis, or the increased excretion of a…”
Background
Tissue Carnitine Deficiency due to Dietary Lysine Deficiency: Triglyceride Accumulation and Concomitant Impairment in Fatty Acid Oxidation
Khan, L., & Bamji, M. S. (1979) · Journal of Nutrition · doi:10.1093/jn/109.1.24 · Cited by 59 · cited 1× · see citing works
“Reduced plasma carnitine levels have been noted in malnourished children [106] and adults [107].”
Background
Structural features of the gene encoding human muscle type carnitine palmitoyltransferase I1
Yamazaki, N., Yamanaka, Y., Hashimoto, Y. K., Shinohara, Y., Shima, A., & Terada, H. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)00561-9 · Cited by 58 · cited 1× · see citing works
“The nucleotide sequences of cDNA and genomic DNA encoding human CPTI have been characterized [45,46].”
Method
Pharmacological stimulation of brain carnitine palmitoyl-transferase-1 decreases food intake and body weight
Aja, S. M., Landree, L. E., Kleman, A. M., Medghalchi, S. M., Vadlamudi, A., McFadden, J. M., Aplasca, A., Hyun, J., Plummer, E. R., Daniels, K., et al. (2007) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.00862.2006 · Cited by 58 · cited 3× · see citing works
“A selective CPT-1 stimulator produced long lasting hypophagia (reduced food intake) and persistent weight loss [75].”
BackgroundInfluential
Turning down lipid oxidation during heavy exercise--what is the mechanism?
Sahlin, K., Sallstedt, E.-K., Bishop, D. J., & Tonkonogi, M. (2008) · PubMed · Cited by 56 · see citing works
Expression and Localization of Carnitine/Organic Cation Transporter OCTN1 and OCTN2 in Ocular Epithelium
Garrett, Q., Xu, S., Simmons, P. A., Vehige, J. G., Flanagan, J., & Willcox, M. D. P. (2008) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.07-1528 · Cited by 55 · cited 1× · see citing works
“Use of carnitine in artificial tears has demonstrated rapid and consistent improvements in signs and symptoms in patients with dry eye [159] suggesting an intrinsic homeostatic role for carnitine in the eye [160].”
BackgroundSelf-citation
Supplementation of L -carnitine Improves Mitochondrial Enzymes in Heart and Skeletal Muscle of Aged Rats
Kumaran, S., Subathra, M., Balu, M., & Panneerselvam, C. (2005) · Experimental Aging Research · doi:10.1080/03610730590882846 · Cited by 54 · cited 1× · see citing works
“Several studies have reported that supplementing rats with both L-carnitine and alpha-lipoic acid halts age-related increases in reactive oxygen species (ROS), lipid peroxidation, protein carbonylation, and DNA strand breaks in heart, skeletal muscle and brain, concomitant with improvement in mitochondrial enzyme and…”
Background
l‐Carnitine and Short Chain Ester in Tears from Patients with Dry Eye
Pescosolido, N., Imperatrice, B., Koverech, A., & Messano, M. (2009) · Optometry and Vision Science · doi:10.1097/opx.0b013e318194e767 · Cited by 54 · cited 1× · see citing works
“Recently, Pescosolido and colleagues [161] evaluated the presence of carnitine in tears of dry eye patients and suggested that the damage incurred on the ocular surface of dry eye patients may, in part, be due to a lack of carnitine in the tear film of these patients relative to the ocular surface cells and suggested…”
Background
Urinary excretion of carnitine in patients with hyperthyroidism and hypothyroidism: Augmentation by thyroid hormone
Maebashi, M., Kawamura, N., Sato, M., Imamura, A., YOSHINAGA, K., & Suzuki, M. (1977) · Metabolism · doi:10.1016/0026-0495(77)90101-9 · Cited by 53 · cited 1× · see citing works
“Hyperthyroid patients exhibit higher urinary carnitine concentrations compared with controls while hypothyroid patients exhibit concomitantly lower levels [88].”
Background
Silent and symptomatic primary carnitine deficiency within the same family due toidentical mutations in the organic cation/carnitine transporter OCTN2
Spiekerkoetter, U., Hüner, G., Baykal, T., Demirkol, M., Durán, M., Wanders, R. J. A., Nezu, J., & Mayatepek, E. (2003) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1025968502527 · Cited by 53 · cited 2× · see citing works
“Several mutations have been described [21,22,25,26,29,31-33].”
Background
Plasma Carnitine Levels and Urinary Carnitine Excretion during Sepsis
Nanni, G., Pittiruti, M., Giovannini, I., Boldrini, G., Ronconi, P., & Castagneto, M. (1985) · Journal of Parenteral and Enteral Nutrition · doi:10.1177/0148607185009004483 · Cited by 52 · cited 1× · see citing works
“A study of plasma and urinary levels of free carnitine and short-chain acyl-carnitines in surgical patients showed that the septic state was associated with increased urinary excretion of free carnitine and lower plasma levels of short-chain acyl-carnitines [101].”
Background
L-Carnitine and Isovaleryl L-Carnitine Fumarate Positively Affect Human Osteoblast Proliferation and Differentiation In Vitro
Colucci, S. C., Mori, G., Vaira, S., Brunetti, G., Greco, G., Mancini, L., Simone, G. M., Sardelli, F., Koverech, A., Zallone, A. Z., et al. (2005) · Calcified Tissue International · doi:10.1007/s00223-004-0147-4 · Cited by 52 · see citing works
Metabolic Treatment with L-Carnitine in Acute Anterior ST Segment Elevation Myocardial Infarction
Tarantini, G., Scrutinio, D., Bruzzi, P., Boni, L., RIZZON, P., & Iliceto, S. (2006) · Cardiology · doi:10.1159/000093131 · Cited by 50 · cited 1× · see citing works
“L-carnitine reduces early mortality but not overall risk of death or heart failure at 6 months [127].”
Background
L-Carnitine in the Treatment of Mild or Moderate Hepatic Encephalopathy
Malaguarnera, M., Pistone, G., Astuto, M., Dell'Arte, S., Finocchiaro, G., Giudice, E. L., & Pennisi, G. (2003) · Digestive Diseases · doi:10.1159/000073347 · Cited by 50 · cited 2× · see citing works
“One suggested mechanism of carnitine action is its reduction of serum ammonia levels leading to improved psychometric measures [63-65].”
BackgroundResult
Age-associated deficit of mitochondrial oxidative phosphorylation in skeletal muscle: Role of carnitine and lipoic acid
Kumaran, S., Panneerselvam, K. S., Shila, S., Sivarajan, K., & Panneerselvam, C. (2005) · Molecular and Cellular Biochemistry · doi:10.1007/s11010-005-8234-z · Cited by 49 · cited 1× · see citing works
“Several studies have reported that supplementing rats with both L-carnitine and alpha-lipoic acid halts age-related increases in reactive oxygen species (ROS), lipid peroxidation, protein carbonylation, and DNA strand breaks in heart, skeletal muscle and brain, concomitant with improvement in mitochondrial enzyme and…”
Background
Clinical varieties of carnitine and carnitine palmitoyltransferase deficiency
Angelini, C. I., Trevisan, C. L., Isaya, G., Pegolo, G., & Vergani, L. (1987) · Clinical Biochemistry · doi:10.1016/s0009-9120(87)80090-5 · Cited by 49 · cited 2× · see citing works
“Its transport within cells of various tissues is limited by their respective uptake capacities [10].”
Background
Effects of l-Acetylcarnitine on Cirrhotic Patients with Hepatic Coma: Randomized Double-Blind, Placebo-Controlled Trial
Malaguarnera, M., Pistone, G., Astuto, M., Vecchio, I., Raffaele, R., Giudice, E. L., & Rampello, L. (2006) · Digestive Diseases and Sciences · doi:10.1007/s10620-006-9187-0 · Cited by 48 · cited 2× · see citing works
“One suggested mechanism of carnitine action is its reduction of serum ammonia levels leading to improved psychometric measures [63-65].”
BackgroundResult
Muscle carnitine levels in neuromuscular disease
Borum, P. R., Broquist, H. P., & Roelofs, R. I. (1977) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(77)90075-2 · Cited by 45 · cited 1× · see citing works
“Patients with Duchenne dystrophy and Becker dystrophy showed lower carnitine levels in muscle biopsies than controls [131] though these levels were higher than in patients suffering primary carnitine deficiency as a result of severe muscle damage [1].”
Background
A new era for carnitine?
Famularo, G., & Simone, C. D. (1995) · Immunology Today · doi:10.1016/0167-5699(95)80159-6 · Cited by 42 · cited 1× · see citing works
“Carnitine levels are reduced in patients suffering Gram-negative sepsis and urinary loss of carnitine is proportional to the degree of injury [96].”
Background
Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose
Ørngreen, M. C., Olsen, D. B., & Vissing, J. (2002) · Neurology · doi:10.1212/wnl.59.7.1046 · Cited by 42 · cited 1× · see citing works
“During sustained low to moderate exercise, fatty acid oxidation increases to become the predominant energy source to muscles [17].”
Background
Carnitine metabolism in patients with chronic renal failure: effect of L-carnitine supplementation.
Wanner, C., Förstner-Wanner, S., Rössle, C., Fürst, P., Schollmeyer, P. J., & Wh, H. (1987) · PubMed · Cited by 42 · see citing works
Acetyl-L-Carnitine in Diabetic Polyneuropathy
Sima, A. A. F. (2007) · CNS Drugs · doi:10.2165/00023210-200721001-00003 · Cited by 41 · see citing works
Primary carnitine deficiency
Stanley, C. A. (1989) · Biomedicine & Pharmacotherapy · doi:10.1016/0753-3322(89)90043-7 · Cited by 38 · see citing works
L‐carnitine supplementation in the dialysis population: Are Australian patients missing out? (Review Article)
Reuter, S. E., Faull, R., & Evans, A. M. (2007) · Nephrology · doi:10.1111/j.1440-1797.2007.00817.x · Cited by 38 · see citing works
Carnitine Deficiency: Primary and Secondary Syndromes
Famularo, G., Matricardi, F., Nucera, E., Santini, G., & Simone, C. D. (1997) · Springer eBooks · doi:10.1007/978-1-4615-6005-0_6 · Cited by 36 · cited 1× · see citing works
“As opposed to PCD, SCD occurs due to, or in association with, other disorders such as liver or kidney disease, defects in fatty acid metabolism, or administration of pharmacological agents such as pivampicillin or valproic acid (discussed below) [27,41,42].”
Background
Pathology of Long-Chain 3-Hydroxyacyl-Coa Dehydrogenase Deficiency Caused by the G1528C Mutation
Tyni, T. A., Rapola, J., Paetau, A. E., Palotie, A. V., & Pihko, H. S. (1997) · Pediatric Pathology & Laboratory Medicine · doi:10.1080/15513819709168585 · Cited by 36 · see citing works
Myocardial infarction and left ventricular remodeling: Results of the CEDIM trial
Colonna, P., & Iliceto, S. (2000) · American Heart Journal · doi:10.1067/mhj.2000.103918 · Cited by 35 · see citing works
Dietary l-carnitine supplementation improves bone mineral density by suppressing bone turnover in aged ovariectomized rats
Hooshmand, S., Balakrishnan, A., Clark, R. M., Owen, K. Q., Koo, S. I., & Arjmandi, B. H. (2008) · Phytomedicine · doi:10.1016/j.phymed.2008.02.026 · Cited by 35 · cited 1× · see citing works
“Using an aging ovariectomized rat model they found supplementation of L-carnitine can influence bone density and slow the rate of bone turnover by slowing bone loss and improving bone microstructural properties through decreasing bone turnover [154].”
Method
Type V collagen regulates the assembly of collagen fibrils in cultures of bovine vascular smooth muscle cells
Kypreos, K. E., Birk, D. E., Trinkaus‐Randall, V., Hartmann, D. J., & Sonenshein, G. E. (2000) · Journal of Cellular Biochemistry · doi:10.1002/1097-4644(20010101)80:1 · Cited by 34 · see citing works
Ophthalmic pathology in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation
Tyni, T. A., Pihko, H. S., & Kivelä, T. (1998) · Current Eye Research · doi:10.1080/02713689808951227 · Cited by 33 · cited 1× · see citing works
“Roomets et al. examined the expression of CPT-1 isoforms in photoreceptor cells and retinal pigment epithelial cells that are known to be affected morphologically and functionally in complete MTP deficiency and deficiency of long-chain 3-hydroxyacyl-CoA hydratase (LCHAD) [ 167 ].”
Background
Myocardial carnitine palmitoyltransferase I as a target for oxidative modification in inflammation and sepsis
Eaton, S. D., Fukumoto, K., Stefanutti, G., Spitz, L., Zammit, V. A., & Pierro, A. (2003) · Biochemical Society Transactions · doi:10.1042/bst0311133 · Cited by 32 · cited 2× · see citing works
“[100] have suggested that inhibition of myocardial CPT I activity may be a common feature of systemic inflammation, or of inflammation localized to the heart.”
Background
Dietary oxidised fat up regulates the expression of organic cation transporters in liver and small intestine and alters carnitine concentrations in liver, muscle and plasma of rats
Koch, A. H., König, B., Luci, S., Stangl, G. I., & Eder, K. (2007) · British Journal Of Nutrition · doi:10.1017/s000711450775691x · Cited by 31 · cited 2× · see citing works
“Oxidized fat was shown to upregulate PPARα and OCNT2 and lead to reduced rate of weight gain compared to controls, indicating an impairment of the feed conversion ratio [112].”
Background
Prevalence of carnitine depletion in critically ill patients with undernutrition
Wennberg, A., Hyltander, A. G., Sjöberg, Å. K., Arfvidsson, B., Sandström, R., Wickström, I., & Lundholm, K. (1992) · Metabolism · doi:10.1016/0026-0495(92)90146-2 · Cited by 31 · cited 1× · see citing works
“There have been varying reports of urinary freecarnitine excretion with PEM as either increasing [108] or decreasing [109].”
Background
Urinary excretion of carnitine in burned patients
Cederblad, G., Larsson, J., Nordström, H., & Schildt, B. E. (1981) · Burns · doi:10.1016/0305-4179(81)90030-9 · Cited by 30 · cited 1× · see citing works
“In relation to burns and wound healing; one study involving 14 patients with severe burns over eight days showed dramatically increased levels of excreted carnitine [ 102 ].”
Background
Phenotypic reversal of the btn1 defects in yeast by chloroquine: A yeast model for Batten disease
Pearce, D. A., Carr, C. J., Das, B., & Sherman, F. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.20.11341 · Cited by 30 · cited 1× · see citing works
“There appears to be an anomalous storage of mitochondrial ATP synthase subunit c that is neither the result of mutation nor enhanced expression of the protein but rather a slower degradation of the mitochondrial ATP synthase in comparison with normal cells [116].”
Background
l-carnitine as an Adjunct Therapy to Percutaneous Coronary Intervention for Non-ST Elevation Myocardial Infarction
Xue, Y., Wang, L., Liu, H., Qi, X.-W., Wang, X., & Ren, H.-Z. (2007) · Cardiovascular Drugs and Therapy · doi:10.1007/s10557-007-6056-9 · Cited by 29 · see citing works
Dose-Dependent Lipotropic Effect of Carnitine in Chronic Alcoholic Rats
Rhew, T. H., & Sachan, D. S. (1986) · Journal of Nutrition · doi:10.1093/jn/116.11.2263 · Cited by 28 · cited 1× · see citing works
“carnitine which is available for acylation [72].”
Characteristics of L-carnitine transport by lactating rat mammary tissue
SHENNAN, D. B., Grant, A., Ramsay, R. R., BURNS, C., & Zammit, V. A. (1998) · Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism · doi:10.1016/s0005-2760(98)00056-3 · Cited by 26 · cited 1× · see citing works
“In one example of a physiological response to osmotic pressure, in early mammary gland milk production osmoregulatory pathways are exploited using asymmetric kinetics to increase the carnitine concentration in milk for suckling neonates who have reduced carnitine stores, even though this results in decreased maternal…”
Background
Carnitine Palmitoyltransferase I and Acyl-CoA Dehydrogenase 9 in Retina: Insights of Retinopathy in Mitochondrial Trifunctional Protein Defects
Roomets, E., Kivelä, T., & Tyni, T. A. (2008) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.07-1094 · Cited by 26 · cited 2× · see citing works
“They further suggest that accumulation of 3-hydroxylated intermediates of long-chain fatty acids may contribute to the pathogenesis of retinopathy in MTP deficiencies [ 164 ].”
Background
In the Rat Brain Acetyl-l-carnitine Treatment Modulates the Expression of Genes Involved in Neuronal Ceroid Lipofuscinosis
Traina, G., Bernardi, R., Cataldo, E., Macchi, M., Durante, M., & Brunelli, M. (2008) · Molecular Neurobiology · doi:10.1007/s12035-008-8038-8 · Cited by 26 · cited 4× · see citing works
“Acetyl-L-carnitine is neuroprotective when administered at supraphysiological concentration [114].”
BackgroundInfluential
Protective effect of l-propionylcarnitine in chronic cyclosporine-a induced nephrotoxicity
Origlia, N., MIGLIORI, M., Panichi, V., Filippi, C., Bertelli, A., Carpi, A., & Giovannini, L. (2005) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2005.06.014 · Cited by 25 · cited 2× · see citing works
“Origlia and colleagues further demonstrated L-PC-associated reduction in lipid hydroperoxide content and morphological abnormalities associated with chronic CyA administration [57].”
Background
The effect of acetyl-l-carnitine administration on persons with Down syndrome
Pueschel, S. M. (2006) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2004.07.009 · Cited by 24 · cited 4× · see citing works
“However acetylL-carnitine was not found to benefit young men suffering Down's Syndrome [117].”
BackgroundMethodResultInfluential
In Experimental Diabetes the Decrease in the Eye of Lens Carnitine Levels is an Early Important and Selective Event
Pessotto, P., Liberati, R., Petrella, O., Romanelli, L., Calvani, M., & Peluso, G. (1997) · Experimental Eye Research · doi:10.1006/exer.1996.0188 · Cited by 24 · cited 1× · see citing works
“[163]) might be related to osmotic stress rather than pathological modification of the eye lens and that chronic aberration of osmotic pressure causes net loss of carnitine which can lead to triggering of cataracts.”
Background
Modulation of carnitine palmitoyltransferase-1 for the treatment of obesity.
Kuhajda, F. P., & Ronnett, G. V. (2007) · PubMed · Cited by 23 · see citing works
Is Oral l-Acyl-Carnitine an Effective Therapy for Hepatic Encephalopathy? Review of the Literature
Shores, N. J., & Keeffe, E. B. (2008) · Digestive Diseases and Sciences · doi:10.1007/s10620-007-0183-9 · Cited by 23 · cited 3× · see citing works
“Although the mechanisms by which carnitine provides neurological protection are unknown, a systematic review of the literature confirmed that L-acyl-carnitine is promising as a safe and effective treatment for HE [58].”
BackgroundInfluential
HMG CoA reductase inhibitor accelarates aging effect on diaphragm mitochondrial respiratory function in rats
Sugiyama, S. (1998) · IUBMB Life · doi:10.1080/15216549800204472 · Cited by 23 · cited 1× · see citing works
“Several studies have reported that supplementing rats with both L-carnitine and alpha-lipoic acid halts age-related increases in reactive oxygen species (ROS), lipid peroxidation, protein carbonylation, and DNA strand breaks in heart, skeletal muscle and brain, concomitant with improvement in mitochondrial enzyme and…”
Background
L-Carnitine and -Lipoic Acid Improve Age-Associated Decline in Mitochondrial Respiratory Chain Activity of Rat Heart Muscle
Sethumadhavan, S., & Chinnakannu, P. (2006) · The Journals of Gerontology Series A · doi:10.1093/gerona/61.7.650 · Cited by 23 · cited 1× · see citing works
“Several studies have reported that supplementing rats with both L-carnitine and alpha-lipoic acid halts age-related increases in reactive oxygen species (ROS), lipid peroxidation, protein carbonylation, and DNA strand breaks in heart, skeletal muscle and brain, concomitant with improvement in mitochondrial enzyme and…”
Background
Nutrition support for glutaric acidemia type I
Yannicelli, S., Rohr, F., & Warman, M. L. (1994) · Journal of the American Dietetic Association · doi:10.1016/0002-8223(94)90245-3 · Cited by 22 · cited 1× · see citing works
“GCDH can be controlled nutritionally and supplementation includes L-carnitine to avoid secondary carnitine depletion [84-87].”
Background
l-Carnitine Fumarate and Isovaleryl-l-Carnitine Fumarate Accelerate the Recovery of Bone Volume/Total Volume Ratio after Experimetally Induced Osteoporosis in Pregnant Mice
Patano, N., Mancini, L., SETTANNI, M., Strippoli, M., Brunetti, G., Greco, G., Tamma, R., Vergari, R., Sardelli, F., Koverech, A., et al. (2008) · Calcified Tissue International · doi:10.1007/s00223-008-9109-6 · Cited by 22 · cited 2× · see citing works
“Patano and colleagues suggest that this decrease in energy availability might compromise osteoblast activity and bone remodeling in an age-related manner [152].”
Background
Vegetarian Diet Affects Genes of Oxidative Metabolism and Collagen Synthesis
Karlic, H., Schuster, D., Varga, F., Klindert, G., Lapin, A., Haslberger, A. G., & Handschur, M. (2008) · Annals of Nutrition and Metabolism · doi:10.1159/000152871 · Cited by 21 · cited 2× · see citing works
“Thus a vegetarian lifestyle has an impact on fat metabolism causing a remarkable stimulation of carnitine uptake [113].”
Background
Debate Forum: Carnitine Supplements Have Not Been Demonstrated as Effective in Patients on Long-Term Dialysis Therapy
Handelman, G. J. (2005) · Blood Purification · doi:10.1159/000089450 · Cited by 19 · cited 1× · see citing works
“Handelman however cautions that evidence for effectiveness of carnitine supplements in dialysis suffers from trials limited in subject number and open labeled, and suggests more rigorous testing is needed [55].”
Background
A Case of Early Diagnosed Carnitine Deficiency Presenting with Respiratory Symptoms
Ergüven, M., Yılmaz, O., Koç, S., Çakı, S., Ayhan, Y. İ., Donmez, M., & Dolunay, G. (2007) · Annals of Nutrition and Metabolism · doi:10.1159/000107675 · Cited by 18 · cited 2× · see citing works
“Cardiomyopathy has also be observed [30].”
Background
Effect of L-Carnitine on the Serum Lipoproteins and HDL-C Subclasses in Hemodialysis Patients
Argani, H., Rahbaninoubar, M., Ghorbanihagjo, A., Golmohammadi, Z., & Rashtchizadeh, N. (2005) · Nephron Clinical Practice · doi:10.1159/000087411 · Cited by 18 · cited 1× · see citing works
“Argani and colleagues showed a decrease in plasma levels of triglyceride (TG) and very low-density lipoprotein, and increases in total high-density lipoprotein cholesterol (HDL-C and HDL2-C) and albumin, in hemodialysis patients upon administration of 500 mg/day carnitine taken orally for 2 months [44].”
Background
Brief report. Plasma and urine carnitine levels supplementation in children with malnutrition
Alp, H. (1999) · Journal of Tropical Pediatrics · doi:10.1093/tropej/45.5.294 · Cited by 15 · see citing works
Rationales for micronutrient supplementation in diabetes
McCarty, M. F., & Rubin, E. J. (1984) · Medical Hypotheses · doi:10.1016/0306-9877(84)90025-2 · Cited by 14 · cited 1× · see citing works
“McCarty and Rubin suggest supplementation of micronutrients including carnitine to aid wound healing in diabetics [103].”
Background
The additive effects of carnitine and ascorbic acid on distally burned dorsal skin flap in rats.
Arslan, E., Başterzi, Y., Aksoy, A., Majka, C., Ünal, Ş., Sarı, A., & Demirkan, F. (2005) · PubMed · Cited by 14 · see citing works
Autopsy case of the neonatal form of carnitine palmitoyltransferase‐II deficiency triggered by a novel disease‐causing mutation del1737C
Semba, S., Yasujima, H., Takano, T., & Yokozaki, H. (2008) · Pathology International · doi:10.1111/j.1440-1827.2008.02250.x · Cited by 13 · cited 1× · see citing works
“One case study found a novel mutation in CPT II (del1737C), an autosomal recessive disease with a distinct phenotype [49].”
Background
Increased blood ammonia in hypoxia during exercise in humans
Casas, H., Murtra, B., Casas, M., Ibáñez, J., Ventura, J. L., Ricart, A., Rodríguez, F. A., Viscor, G., Palacios, L., Pagés, T., et al. (2001) · Journal of Physiology and Biochemistry · doi:10.1007/bf03179824 · Cited by 12 · cited 1× · see citing works
“This is supported by other studies that showed a protective effect of L-carnitine against ammonia-evoked encephalopathy in cirrhotic patients, with ALC administration improving neurological symptoms and plasmatic parameters in cirrhotic patients with hepatic coma [60-67].”
Result
Protective effect of L-propionylcarnitine on cyclosporine-induced nephrotoxicity.
Bertelli, A. A. E., Giovannini, L., Palla, R., MIGLIORI, M., Panichi, V., & Andreini, B. (1995) · PubMed · Cited by 12 · see citing works
Association for Research in Vision and Ophthalmology (ARVO) Annual Meeting
Waring, G. O., IV (1990) · Journal of Refractive Surgery · doi:10.3928/1081-597x-19900501-18 · Cited by 11 · see citing works
Effect of endocrine pancreas allotransplantation on diabetic nerve dysfunction
Tze, W. J., Sima, A. A. F., & Tai, J. (1985) · Metabolism · doi:10.1016/0026-0495(85)90021-6 · Cited by 10 · cited 1× · see citing works
“In both experimental models and human diabetic subjects, there is an initial metabolic phase that is responsive to metabolic corrections [90,91].”
Background
Carnitine worsens both injury and recovery of contractile function after transient ischemia in perfused rat heart
Díaz, R., Lorita, J., Soley, M., & Ramı́rez, I. (2008) · Journal of Physiology and Biochemistry · doi:10.1007/bf03168229 · Cited by 9 · cited 3× · see citing works
“[122] demonstrated that carnitine worsened the recovery of contractile function in transient ischemia.”
BackgroundInfluential
C-peptide and diabetic neuropathy
Sima, A. A. F. (2003) · Expert Opinion on Investigational Drugs · doi:10.1517/eoid.12.9.1471.21816 · Cited by 9 · see citing works
Substitutions of Three Amino Acids in Human Heart/Muscle Type Carnitine Palmitoyltransferase I Caused by Single Nucleotide Polymorphisms
Yamazaki, N., Matsuo, T., Kurata, M., Suzuki, M., Fujiwaki, T., Yamaguchi, S., Terada, H., & Shinohara, Y. (2007) · Biochemical Genetics · doi:10.1007/s10528-007-9129-3 · Cited by 8 · cited 1× · see citing works
“However, a relationship between disease and mutation of the human CPTI gene has not been reported [47].”
Background
L-carnitine in dialysed patients: the choice of dosage regimen.
Bérard, E., Iordache, A., Barrillon, D., & Bayle, J. (1995) · PubMed · Cited by 8 · see citing works
CARNITINE DEPRIVATION ADVERSELY AFFECTS CARDIOVASCULAR RESPONSE TO BACTERIAL ENDOTOXIN (LPS) IN THE ANESTHETIZED NEONATAL PIG
Penn, D., Zhang, L., Bobrowski, P. J., Quinn, M., Liu, X., & McDonough, K. H. (1998) · Shock · doi:10.1097/00024382-199811000-00012 · Cited by 7 · cited 1× · see citing works
“It has been further suggested that carnitine deficiency might negatively impact cardiac function which might in turn, further contribute to the outcome of patients suffering sepsis [97-99].”
Background
CARNITINE DEPRIVATION ADVERSELY AFFECTS CARDIAC PERFORMANCE IN THE LIPOPOLYSACCHARIDE- AND HYPOXIA/ REOXYGENATION-STRESSED PIGLET HEART
Penn, D., Zhang, L., Bobrowski, P. J., Quinn, M., & McDonough, K. H. (1999) · Shock · doi:10.1097/00024382-199902000-00009 · Cited by 7 · cited 1× · see citing works
“It has been further suggested that carnitine deficiency might negatively impact cardiac function which might in turn, further contribute to the outcome of patients suffering sepsis [97-99].”
Background
L-carnitine administration reverses acute mental status changes in a chronic hemodialysis patient with hepatitis C infection.
DaVanzo, W., & Ullian, M. E. (2002) · PubMed · Cited by 5 · see citing works
Mutations in the X-linked pyruvate dehydrogenase (E1) α subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency
Lissens, W., Meırleır, L. D., Seneca, S., Liebaers, I., Brown, G. K., Brown, R. M., Ito, M., Naito, E., Kuroda, Y., Kerr, D. S., et al. (2000) · Human Mutation · doi:10.1002/(sici)1098-1004(200003)15:3 · Cited by 5 · see citing works
Fatty Acid Oxidation Disorders
Tyni, T. A., & Turnbull, D. M. (2001) · Encyclopedia of Life Sciences · doi:10.1038/npg.els.0003067 · Cited by 5 · see citing works
Pathogenetic mechanisms of acute hepatic encephalopathy.
Mullen, K. D., & Gacad, R. (1994) · PubMed · Cited by 4 · see citing works
Ophthalmologic findings in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation - A new type of hereditary metabolic chorioretinopathy
Tyni, T. A., Kivelä, T., Lappi, M., Summanen, P. A., Nikoskelainen, E. K., & Pihko, H. S. (1998) · International Ophthalmology · Cited by 2 · see citing works
June-November 2002
(2002) · Archives of Neurology · doi:10.1001/archneur.59.6.1046 · Cited by 1 · see citing works

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Rebouche C Shils M, Shike M, Ross A, et al Carnitine: Modern Nutrition in Health and Disease 2006 Philadelphia: Lippincott, Williams and Wilkins 537 544

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Revisiting Fluorescent Calixarenes: From Molecular Sensors to Smart Materials
Kumar, R., Sharma, A. K., Singh, H., Suating, P., Kim, H. S., Sunwoo, K., Shim, I., Gibb, B. C., & Kim, J. S. (2019) · Chemical Reviews · doi:10.1021/acs.chemrev.8b00605 · Cited by 541 · see citing works
Role of Hyperosmolarity in the Pathogenesis and Management of Dry Eye Disease: Proceedings of the OCEAN Group Meeting
Baudouin, C., Aragona, P., Messmer, E. S. B. M., Tomlinson, A., Calonge, M., Boboridis, K. G., Akova, Y. A., Geerling, G., Labétoulle, M., & Rolando, M. (2013) · The Ocular Surface · doi:10.1016/j.jtos.2013.07.003 · Cited by 511 · see citing works
Trimethylamine N -Oxide, the Microbiome, and Heart and Kidney Disease
Zeisel, S. H., & Warrier, M. (2017) · Annual Review of Nutrition · doi:10.1146/annurev-nutr-071816-064732 · Cited by 475 · see citing works
“Animal products like meat, fish, poultry, and milk (whey fraction) are highest in carnitine, whereas plants contain little carnitine (42).”
Background
Trimethylamine and Trimethylamine N-Oxide, a Flavin-Containing Monooxygenase 3 (FMO3)-Mediated Host-Microbiome Metabolic Axis Implicated in Health and Disease
Fennema, D., Phillips, I. R., & Shephard, E. A. (2016) · Drug Metabolism and Disposition · doi:10.1124/dmd.116.070615 · Cited by 355 · see citing works
“Indeed, carnitine has proven efficacious for treatment of cardiovascular disease (Flanagan et al., 2010).”
Background
Effect of ethanol on lipid metabolism
You, M., & Arteel, G. E. (2019) · Journal of Hepatology · doi:10.1016/j.jhep.2018.10.037 · Cited by 357 · see citing works
Effect of medium chain fatty acid in human health and disease
Roopashree, P. G., Shetty, S. S., & Kumari, S. (2021) · Journal of Functional Foods · doi:10.1016/j.jff.2021.104724 · Cited by 234 · see citing works
Exosomes in inflammation and role as biomarkers
Console, L., Scalise, M., & Indiveri, C. (2018) · Clinica Chimica Acta · doi:10.1016/j.cca.2018.11.009 · Cited by 216 · see citing works
Integrative Personal Omics Profiles during Periods of Weight Gain and Loss
Piening, B., Zhou, W., Contrepois, K., Röst, H. L., Gu, G. J., Mishra, T., Hanson, B., Bautista, E. J., Leopold, S. R., Yeh, C. Y., et al. (2018) · Cell Systems · doi:10.1016/j.cels.2017.12.013 · Cited by 210 · see citing works
“In the case of N6-trimethyllysine, it is a precursor for L-carnitine biosynthesis, which improves glucose tolerance, increases total energy expenditure in obesity (Flanagan et al., 2010), and decreases cardiovascular risks (Koeth et al.”
Background
Carnitine in bacterial physiology and metabolism
Meadows, J. A., & Wargo, M. J. (2015) · Microbiology · doi:10.1099/mic.0.000080 · Cited by 192 · see citing works
“Later, it was discovered that carnitine can be synthesized in mammals and is now considered to be a quasi-nutrient or conditionally essential nutrient (Flanagan et al., 2010), as neonates have reduced biosynthesis and rely on placental transfer of carnitine in utero and exogenous sources after birth (Combs, 2012).”
Gut microbiota-derived metabolites in obesity: a systematic review
Ejtahed, H., Angoorani, P., Soroush, A., Hasani‐Ranjbar, S., Siadat, S. D., & Larijani, B. A. (2020) · Bioscience of Microbiota Food and Health · doi:10.12938/bmfh.2019-026 · Cited by 187 · see citing works
Toward a Predictive Model of Alzheimer’s Disease Progression Using Capillary Electrophoresis–Mass Spectrometry Metabolomics
Ibáñez, C., Simó, C., Martín‐Álvarez, P. J., Kivipelto, M., Winblad, B. G., Cedazo-Mı́nguez, Á., & Cifuentes, A. (2012) · Analytical Chemistry · doi:10.1021/ac301243k · Cited by 166 · see citing works
Function, Detection and Alteration of Acylcarnitine Metabolism in Hepatocellular Carcinoma
Li, S., Gao, D., & Jiang, Y. (2019) · Metabolites · doi:10.3390/metabo9020036 · Cited by 168 · see citing works
Identification of TMAO-producer phenotype and host–diet–gut dysbiosis by carnitine challenge test in human and germ-free mice
Wu, W., Chen, C., Liu, P., Panyod, S., Liao, B., Chen, P.-C., Kao, H., Kuo, H., Kuo, C., Chiu, T. H. T., et al. (2018) · Gut · doi:10.1136/gutjnl-2018-317155 · Cited by 156 · see citing works
Trimethylamine N-Oxide in Relation to Cardiometabolic Health—Cause or Effect?
Papandreou, C., Moré, M. I., & Bellamine, A. (2020) · Nutrients · doi:10.3390/nu12051330 · Cited by 150 · see citing works
l-Carnitine Supplementation in Recovery after Exercise
Fielding, R. A., Riede, L., Lugo, J. P., & Bellamine, A. (2018) · Nutrients · doi:10.3390/nu10030349 · Cited by 146 · see citing works
Translating the basic knowledge of mitochondrial functions to metabolic therapy: role of L-carnitine
Marcovina, S. M., Sirtori, C. R., Peracino, A. P., Gheorghiade, M., Borum, P. R., Remuzzi, G., & Ardehali, H. (2012) · Translational research · doi:10.1016/j.trsl.2012.10.006 · Cited by 146 · see citing works
“Carnitine supplementation may be of additional benefit when treating obesity, potentially because of its effects in improving glucose intolerance and total energy expenditure.(62) Capaldo et al observed that whole body glucose use is acutely increased by L-carnitine infusion in insulinresistant patients with type 2 di…”
Effect of Maternal Obesity on Placental Lipid Metabolism
Calabuig-Navarro, V., Haghiac, M., Minium, J., Glazebrook, P. A., Ranasinghe, G. C., Hoppel, C. L., Mouzon, S. H., Catalano, P., & O’Tierney-Ginn, P. (2017) · Endocrinology · doi:10.1210/en.2017-00152 · Cited by 144 · see citing works
“, carnitine palmitoyltransferase (CPT)1] that synthesize these acylcarnitines (ACs) regulates b-oxidation rates (15, 16).”
Background
A common X-linked inborn error of carnitine biosynthesis may be a risk factor for nondysmorphic autism
Celestino-Soper, P. B. S., Violante, S., Crawford, E. L., Luo, R., Lionel, A. C., Delaby, E., Cai, G., Sadikovic, B., Lee, K., Lo, C., et al. (2012) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1120210109 · Cited by 139 · see citing works
“American Journal of Biochemistry and Biotechnology 4(2):198–207.”
BackgroundInfluential
Effects of L-carnitine supplementation on oxidative stress and antioxidant enzymes activities in patients with coronary artery disease: a randomized, placebo-controlled trial
Lee, B., Lin, J.-S., Lin, Y., & Lin, P. (2014) · Nutrition Journal · doi:10.1186/1475-2891-13-79 · Cited by 134 · see citing works
“LC facilitates β-oxidation of long-chain fatty acids, participates in metabolism of branched chain amino acids, and stabilizes cellular membranes [4,5].”
Background
The role of antioxidants and other agents in alleviating hyperglycemia mediated oxidative stress and injury in liver
Dey, A., & Lakshmanan, J. (2013) · Food & Function · doi:10.1039/c3fo30317a · Cited by 133 · see citing works
Prospective analysis of circulating metabolites and breast cancer in EPIC
His, M., Viallon, V., Dossus, L., Gicquiau, A., Achaintre, D., Scalbert, A., Ferrari, P., Romieu, I., Onland‐Moret, N. C., Weiderpass, E., et al. (2019) · BMC Medicine · doi:10.1186/s12916-019-1408-4 · Cited by 130 · see citing works
Carnitine Inborn Errors of Metabolism
Almannai, M., Alfadhel, M., & El‐Hattab, A. W. (2019) · Molecules · doi:10.3390/molecules24183251 · Cited by 130 · see citing works
Metabolomics and Lipidomics of Ischemic Stroke
Au, A. (2018) · Advances in clinical chemistry · doi:10.1016/bs.acc.2018.02.002 · Cited by 118 · see citing works
The effect of FASN inhibition on the growth and metabolism of a cisplatin‐resistant ovarian carcinoma model
Papaevangelou, E., de Almeida, G. S., Box, C., deSouza, N. M., & Chung, Y. (2018) · International Journal of Cancer · doi:10.1002/ijc.31392 · Cited by 119 · see citing works
Proteomic and metabonomic biomarkers for hepatocellular carcinoma: a comprehensive review
Kimhofer, T., Fye, H. K. S., Taylor-Robinson, S. D., Thursz, M. R., & Holmes, E. C. (2015) · British Journal of Cancer · doi:10.1038/bjc.2015.38 · Cited by 117 · see citing works
“…and HCC, increased urinary-free carnitine may indicate kidney dysfunction, instead of being specific for HCC. Increased urinary acylcarnitines (esterified acyl groups with carnitine) have been reported in specific FFA oxidation disturbances and after extensive workout (Flanagan et al, 2010).”
Background
Carnitine in Human Muscle Bioenergetics: Can Carnitine Supplementation Improve Physical Exercise?
Gnoni, A., Longo, S., Gnoni, G. V., & Giudetti, A. M. (2020) · Molecules · doi:10.3390/molecules25010182 · Cited by 118 · see citing works
Functional Foods: Exploring the Health Benefits of Bioactive Compounds from Plant and Animal Sources
Dixit, V., Kamal, S. W. J., Chole, P. B., Dayal, D., Chaubey, K. K., Pal, A. K., Xavier, J., Manjunath, B. T., & Bachheti, R. K. (2023) · Journal of Food Quality · doi:10.1155/2023/5546753 · Cited by 115 · see citing works
Serum metabolomic profiling and incident CKD among African Americans.
Yu, B., Zheng, Y., Nettleton, J., Alexander, D. C., Coresh, J., & Boerwinkle, E. (2014) · American Society of Nephrology. Clinical Journal · doi:10.2215/cjn.11971113 · Cited by 102 · see citing works
“Carnitine is synthesized from lysine and methionine, and kidney plays an important role in its biosynthesis (35).”
Background
l-carnitine: Nutrition, pathology, and health benefits
Alhasaniah, A. H. (2022) · Saudi Journal of Biological Sciences · doi:10.1016/j.sjbs.2022.103555 · Cited by 113 · see citing works
Metabolomic profiling reveals amino acid and carnitine alterations as metabolic signatures in psoriasis
Chen, C., Hou, G., Zeng, C., Ren, Y., Chen, X., & Peng, C. (2020) · Theranostics · doi:10.7150/thno.51154 · Cited by 106 · see citing works
“Furthermore, carnitine has been shown to have important anti-inflammatory, antioxidant, lipidlowering, and organ-supporting properties [20, 22, 25].”
Carnitine derivatives
Malaguarnera, M. (2012) · Current Opinion in Gastroenterology · doi:10.1097/mog.0b013e3283505a3b · Cited by 105 · see citing works
Impacts of Heat Stress on Rabbit Immune Function, Endocrine, Blood Biochemical Changes, Antioxidant Capacity and Production Performance, and the Potential Mitigation Strategies of Nutritional Intervention
Liang, Z., Chen, F., Park, S., Balasubramanian, B., & Liu, W. (2022) · Frontiers in Veterinary Science · doi:10.3389/fvets.2022.906084 · Cited by 104 · see citing works
Membrane transporters in a human genome-scale metabolic knowledgebase and their implications for disease
Sahoo, S., Aurich, M. K., Jónsson, J. J., & Thiele, I. (2014) · Frontiers in Physiology · doi:10.3389/fphys.2014.00091 · Cited by 103 · see citing works
Efficacy and Safety of L-Carnitine Treatment for Chronic Heart Failure: A Meta-Analysis of Randomized Controlled Trials
Song, X., Qu, H., Yang, Z., Rong, J., Cai, W., & Zhou, H. (2017) · BioMed Research International · doi:10.1155/2017/6274854 · Cited by 102 · see citing works
“Furthermore, noteworthy is that L-C exerts cardioprotective effects through alternative mechanisms, such as oxidative stress [35], nitric oxide [36], arterial hypertension, cardiac inflammation and fibrosis [37– 39], and interstitial remodeling [40], as well as by improving endothelial function [41].”
Background
Biochemistry of human tear film: A review
Masoudi, S. (2022) · Experimental Eye Research · doi:10.1016/j.exer.2022.109101 · Cited by 102 · see citing works
Gastrointestinal and nutritional issues in joint hypermobility syndrome/ehlers–danlos syndrome, hypermobility type
Castori, M., Morlino, S., Pascolini, G., Blundo, C., & Grammatico, P. (2015) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31431 · Cited by 98 · see citing works
“Beneficial effect on tear composition and dry eye may be also obtained by regular carnitin intake, as previously reported for fatigue [Flanagan et al., 2010], as well as vitamin A.”
Background
Pregnancy-Induced Metabolic Phenotype Variations in Maternal Plasma
Luan, H., Meng, N., Liu, P., Feng, Q., Lin, S., Fu, J. X., Davidson, R., Chen, X., Rao, W., Chen, F., et al. (2014) · Journal of Proteome Research · doi:10.1021/pr401068k · Cited by 94 · see citing works
L‐Carnitine Suppresses Loss of Skeletal Muscle Mass in Patients With Liver Cirrhosis
Ohara, M., Ogawa, K., Suda, G., Kimura, M., Maehara, O., Shimazaki, T., Suzuki, K., Nakamura, A., Umemura, M., Izumi, T., et al. (2018) · Hepatology Communications · doi:10.1002/hep4.1207 · Cited by 93 · see citing works
“Aspartate aminotransferase, IU/L 35 (14-88) 41.”
Influential
Antiinflammatory effects of l-carnitine supplementation (1000 mg/d) in coronary artery disease patients
Lee, B., Lin, J.-S., Lin, Y., & Lin, P. (2014) · Nutrition · doi:10.1016/j.nut.2014.10.001 · Cited by 89 · see citing works
“LC assists in the b-oxidation of long-chain fatty acids and transports fatty acids into the mitochondrial matrix [6, 7].”
Background
The role of nutraceuticals for the treatment of non‐alcoholic fatty liver disease
Ben, M. D., Polimeni, L., Baratta, F., Pastori, D., & Angelico, F. (2016) · British Journal of Clinical Pharmacology · doi:10.1111/bcp.12899 · Cited by 88 · see citing works
OCTN: A Small Transporter Subfamily with Great Relevance to Human Pathophysiology, Drug Discovery, and Diagnostics
Pochini, L., Galluccio, M., Scalise, M., Console, L., & Indiveri, C. (2018) · SLAS DISCOVERY · doi:10.1177/2472555218812821 · Cited by 83 · see citing works
Effects of l -Carnitine, Erythritol and Betaine on Pro-inflammatory Markers in Primary Human Corneal Epithelial Cells Exposed to Hyperosmotic Stress
Hua, X., Su, Z., Deng, R., Lin, J., Li, D., & Pflugfelder, S. C. (2014) · Current Eye Research · doi:10.3109/02713683.2014.957776 · Cited by 83 · see citing works
“L-Carnitine, erythritol and/or betaine have been reported to serve as osmoprotectants for hyperosmotic stress(20,23,51) and inflammatory conditions.(51,52) L-Carnitine therapy has been shown considerable promise in addressing a variety of disease states that involve both primary and secondary carnitine deficiency, car…”
Background
Efficacy of Osmoprotectants on Prevention and Treatment of Murine Dry Eye
Chen, W., Zhang, X., Li, J., Wang, Y., Chen, Q., Hou, C., & Garrett, Q. (2013) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.13-12081 · Cited by 81 · see citing works
Self-citation
Combined metabolic activators improve cognitive functions in Alzheimer’s disease patients: a randomised, double-blinded, placebo-controlled phase-II trial
Yulug, B., Altay, O., Li, X., Hanoglu, L., Cankaya, S., Lam, S., Velioglu, H. A., Yang, H., Coskun, E., Idil, E., et al. (2023) · Translational Neurodegeneration · doi:10.1186/s40035-023-00336-2 · Cited by 79 · see citing works
“These findings fit well with recent human data showing that severe disturbances in carnitine metabolism frequently occur in individuals with AD, in association with severe mitochondrial dysfunction [116, 117].”
Result
Characteristics of Selected Antioxidative and Bioactive Compounds in Meat and Animal Origin Products
Kulczyński, B., Sidor, A., & Gramza‐Michałowska, A. (2019) · Antioxidants · doi:10.3390/antiox8090335 · Cited by 80 · see citing works
“In vegetarians, who are adapted to foodstuffs poor in carnitine, its availability is higher (66–86%) than in the individuals whose diet contains meat (54–72%) [83].”
Background
The PPAR–microbiota–metabolic organ trilogy to fine‐tune physiology
Oh, H. Y. P., Visvalingam, V., & Wahli, W. (2019) · The FASEB Journal · doi:10.1096/fj.201802681rr · Cited by 78 · see citing works
Untargeted Metabolomics for Autism Spectrum Disorders: Current Status and Future Directions
Glinton, K. E., & Elsea, S. H. (2019) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2019.00647 · Cited by 75 · see citing works
“In humans, approximately 75% of carnitine is obtained from the diet, with the remainder synthesized endogenously from the amino acids lysine and methionine (135).”
Background
Metabolic Perturbations from Step Reduction in Older Persons at Risk for Sarcopenia: Plasma Biomarkers of Abrupt Changes in Physical Activity
Saoi, M., Li, A., McGlory, C., Stokes, T., von Allmen, M., Phillips, S. M., & Britz‐McKibbin, P. (2019) · Metabolites · doi:10.3390/metabo9070134 · Cited by 75 · see citing works
Protective Effects of L-Carnitine Against Oxidative Injury by Hyperosmolarity in Human Corneal Epithelial Cells
Hua, X., Deng, R., Li, J., Chi, W., Su, Z., Lin, J., Pflugfelder, S. C., & Li, D. (2015) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.14-16247 · Cited by 73 · see citing works
A High-Performing Plasma Metabolite Panel for Early-Stage Lung Cancer Detection
Zhang, L., Zheng, J., Ahmed, R., Huang, G., Reid, J., Mandal, R., Maksymuik, A., SITAR, D. S., Tappia, P. S., Ramjiawan, B., et al. (2020) · Cancers · doi:10.3390/cancers12030622 · Cited by 73 · see citing works
“Future validation studies involving larger cohorts, additional clinical parameters, and the inclusion of patients with other lung diseases as negative controls are being undertaken.”
Neuroprotective Effects of Pre-Treament with l-Carnitine and Acetyl-l-Carnitine on Ischemic Injury In Vivo and In Vitro
Zhang, R., Zhang, H., Zhang, Z., Wang, T., Jingya, N., Cui, D., & Xu, S. (2012) · International Journal of Molecular Sciences · doi:10.3390/ijms13022078 · Cited by 73 · see citing works
“They have wide roles in brain metabolism [7].”
Background
Hepatocellular carcinoma: Review of disease and tumor biomarkers
Kim, J. U., Shariff, M. I. F., Crossey, M. M. E., Gómez‐Romero, M., Holmes, E. C., Cox, I. J., Fye, H. K. S., Njie, R., & Taylor-Robinson, S. D. (2016) · World Journal of Hepatology · doi:10.4254/wjh.v8.i10.471 · Cited by 72 · see citing works
Untargeted plasma and tissue metabolomics in rats with chronic kidney disease given AST-120
Velenosi, T. J., Hennop, A., Feere, D. A., Tieu, A., Kucey, A. S., Kyriacou, P., McCuaig, L. E., Nevison, S. E., Kerr, M. A., & Urquhart, B. L. (2016) · Scientific Reports · doi:10.1038/srep22526 · Cited by 72 · see citing works
Flavin-containing monooxygenase 3 (FMO3): genetic variants and their consequences for drug metabolism and disease
Phillips, I. R., & Shephard, E. A. (2019) · Xenobiotica · doi:10.1080/00498254.2019.1643515 · Cited by 71 · see citing works
“…with biomarkers of cardiovascular disease (Miller et al., 2014) or that its effect was protective (Fukami et al., 2015) and carnitine, a dietary precursor of trimethylamine, has proven efficacious for treatment of cardiovascular disease (DiNicolantonio et al., 2013; Flanagan et al., 2010).”
Background
Primary Carnitine Deficiency and Cardiomyopathy
Fu, L., Huang, M., & Chen, S. (2013) · Korean Circulation Journal · doi:10.4070/kcj.2013.43.12.785 · Cited by 70 · see citing works
Oolong Tea Extract and Citrus Peel Polymethoxyflavones Reduce Transformation of l -Carnitine to Trimethylamine-N-Oxide and Decrease Vascular Inflammation in l -Carnitine Feeding Mice
Chen, P., Li, S., Koh, Y., Wu, J.-C., Yang, M., Ho, C., & Pan, M. (2019) · Journal of Agricultural and Food Chemistry · doi:10.1021/acs.jafc.9b03092 · Cited by 69 · see citing works
Molecular Analysis of Model Gut Microbiotas by Imaging Mass Spectrometry and Nanodesorption Electrospray Ionization Reveals Dietary Metabolite Transformations
Rath, C. M. B., Alexandrov, T., Higginbottom, S. K., Song, J., Milla, M. E., Fischbach, M. A., Sonnenburg, J. L., & Dorrestein, P. C. (2012) · Analytical Chemistry · doi:10.1021/ac302039u · Cited by 68 · see citing works
Specific gut microbial, biological, and psychiatric profiling related to binge eating disorders: A cross-sectional study in obese patients
Leyrolle, Q., Cserjési, R., Mulders, M. D. G. H., Zamariola, G., Hiel, S., Gianfrancesco, M. A., Rodriguez, J., Portheault, D., Amadieu, C., Leclercq, S., et al. (2020) · Clinical Nutrition · doi:10.1016/j.clnu.2020.09.025 · Cited by 69 · see citing works
Pharmacometabolomics of l -Carnitine Treatment Response Phenotypes in Patients with Septic Shock
Puskarich, M. A., Finkel, M. A., Karnovsky, A., Jones, A. E., Trexel, J., Harris, B. N., & Stringer, K. A. (2014) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201409-415oc · Cited by 66 · see citing works
Oxidative stress and altered lipid metabolism in Friedreich ataxia
Tamarit, J., Òbis, È., & Ros, J. (2016) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2016.06.007 · Cited by 65 · see citing works
Role of Carnitine in Non-alcoholic Fatty Liver Disease and Other Related Diseases: An Update
Li, N., & Zhao, H. (2021) · Frontiers in Medicine · doi:10.3389/fmed.2021.689042 · Cited by 79 · see citing works
“These drugs or diseases increase the excretion of carnitine in the form of acylcarnitine in urine, reduce renal tubule reabsorption and endogenous biosynthesis, and inhibit carnitine transporters, resulting in increased carnitine consumption (73, 74).”
Background
Rapidly declining skeletal muscle mass predicts poor prognosis of hepatocellular carcinoma treated with transcatheter intra-arterial therapies
Kobayashi, T., Kawai, H., Nakano, O., Abe, S., Kamimura, H., Sakamaki, A., Kamimura, K., Tsuchiya, A., Takamura, M., Yamagiwa, S., et al. (2018) · BMC Cancer · doi:10.1186/s12885-018-4673-2 · Cited by 63 · see citing works
“In addition, patients with impaired liver function are often deficient in vitamin D or carnitine, which might cause skeletal muscle atrophy [38, 39].”
Acetyl-L-carnitine improves cognitive functions in severe hepatic encephalopathy: a randomized and controlled clinical trial
Malaguarnera, M., Vacante, M., Motta, M., Giordano, M., Malaguarnera, G., Bella, R., Nunnari, G., Rampello, L., & Pennisi, G. (2011) · Metabolic Brain Disease · doi:10.1007/s11011-011-9260-z · Cited by 61 · see citing works
“Data suggest that despite fluctuations in carnitine concentration due to its osmolytic pressure changes, carnitine maintains its energy production capacities and often osmolytic gradients can be harnessed for energy (Peluso et al. 2000; Flanagan et al. 2010).”
Background
Global Metabolomic Identification of Long-Term Dose-Dependent Urinary Biomarkers in Nonhuman Primates Exposed to Ionizing Radiation
Pannkuk, E. L., Laiakis, E. C., Authier, S., Wong, K. K.-W., & Fornace, A. J. (2015) · Radiation Research · doi:10.1667/rr14091.1 · Cited by 62 · see citing works
Carnitine deficiency in chronic critical illness
Bonafé, L., Berger, M. M., Que, Y., & Mechanick, J. I. (2014) · Current Opinion in Clinical Nutrition & Metabolic Care · doi:10.1097/mco.0000000000000037 · Cited by 60 · see citing works
Protective action of l-carnitine on cardiac mitochondrial function and structure against fatty acid stress
Oyanagi, E., Yano, H., Uchida, M., Utsumi, K., & Sasaki, J. (2011) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2011.07.039 · Cited by 60 · see citing works
Serum metabolomic profiles suggest influence of sex and oral contraceptive use.
Ruoppolo, M., Campesi, I., Scolamiero, E., Pecce, R., Caterino, M., Cherchi, S., Mercuro, G. G., Tonolo, G., & Franconi, F. (2014) · Europe PMC (PubMed Central) · Cited by 60 · see citing works
Acetyl-L-carnitine in hepatic encephalopathy
Malaguarnera, M. (2013) · Metabolic Brain Disease · doi:10.1007/s11011-013-9376-4 · Cited by 58 · see citing works
Selenium and L-Carnitine Ameliorate Reproductive Toxicity Induced by Cadmium in Male Mice
Alharthi, W. A., Hamza, R. Z., El-Mahdi, M. M., Abu‐El‐Zahab, H. S. H., & Saleh, H. M. (2019) · Biological Trace Element Research · doi:10.1007/s12011-019-02016-7 · Cited by 58 · see citing works
Application of LC-MS-based metabolomics method in differentiating septic survivors from non-survivors
Liu, Z., Yin, P., Amathieu, R., Savarin, P., & Xu, G. (2016) · Analytical and Bioanalytical Chemistry · doi:10.1007/s00216-016-9845-9 · Cited by 57 · see citing works
“Furthermore, we calculated the ratio of carnitine C 2:0 and carnitine which reflects the extent of the betaoxidation [32].”
Method
Randomised clinical trial: oral taurine supplementation versus placebo reduces muscle cramps in patients with chronic liver disease
Vidot, H., Cvejic, E., Carey, S., Strasser, S., Mccaughan, G., Allman-Farinelli, M., & Shackel, N. (2018) · Alimentary Pharmacology and Therapeutics · doi:10.1111/apt.14950 · Cited by 50 · see citing works
“It is involved in the maintenance of cell volume and fluid balance in all tissues.(19) Adverse side effects attributed to carnitine supplementation in humans may include nausea, vomiting, diarrhoea, cramps, muscle weakness, and an abnormal body odour.”
Background
Metabolomic Evidence for Peroxisomal Dysfunction in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome
Che, X., Brydges, C. R., Yu, Y., Price, A., Joshi, S., Roy, A., Lee, B., Barupal, D. K., Cheng, A., Palmer, D. M., et al. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23147906 · Cited by 51 · see citing works
Rapid quantitative analysis of carnitine and acylcarnitines by ultra-high performance–hydrophilic interaction liquid chromatography–tandem mass spectrometry
Kivilompolo, M., Öhrnberg, L., Orešič, M., & Hyötyläinen, T. (2013) · Journal of Chromatography A · doi:10.1016/j.chroma.2012.12.073 · Cited by 54 · see citing works
Effect of L‐carnitine on the synthesis of nitric oxide in RAW 264·7 murine macrophage cell line
Koç, A., Özkan, T., Karabay, A. Z., Sunguroğlu, A., & Aktan, F. (2011) · Cell Biochemistry and Function · doi:10.1002/cbf.1807 · Cited by 54 · see citing works
The effects of graded levels of calorie restriction: IX. Global metabolomic screen reveals modulation of carnitines, sphingolipids and bile acids in the liver of C57BL/6 mice
Green, C. L., Mitchell, S. E., Derous, D., Wang, Y., Chen, L., Han, J. J., Promislow, D., Lusseau, D., Douglas, A., & Speakman, J. R. (2017) · Aging Cell · doi:10.1111/acel.12570 · Cited by 54 · see citing works
Green feed increases antioxidant and antineoplastic activity of buffalo milk: A globally significant livestock
Salzano, A., Neglia, G., D’Onofrio, N., Balestrieri, M. L., Limone, A., Cotticelli, A., Marrone, R., Anastasio, A., D’Occhio, M. J., & Campanile, G. (2020) · Food Chemistry · doi:10.1016/j.foodchem.2020.128669 · Cited by 54 · see citing works
Carnitine transporter CT2 (SLC22A16) is over-expressed in acute myeloid leukemia (AML) and target knockdown reduces growth and viability of AML cells
Wu, Y., Hurren, R., MacLean, N., Gronda, M., Jitkova, Y., Sukhai, M. A., Minden, M. D., & Schimmer, A. D. (2015) · APOPTOSIS · doi:10.1007/s10495-015-1137-x · Cited by 54 · see citing works
“dogenously from lysine and methionine in the kidney, liver and brain [3, 4].”
Nutrients and micronutrients at risk during renal replacement therapy: a scoping review
Berger, M. M., Broman, M., Forni, L. G., Ostermann, M., Waele, E. D., & Wischmeyer, P. E. (2021) · Current Opinion in Critical Care · doi:10.1097/mcc.0000000000000851 · Cited by 53 · see citing works
Carnitine therapy for the treatment of metabolic syndrome and cardiovascular disease: Evidence and controversies
Johri, A. M., Heyland, D. K., Hétu, M., Crawford, B., & Spence, J. D. (2014) · Nutrition Metabolism and Cardiovascular Diseases · doi:10.1016/j.numecd.2014.03.007 · Cited by 53 · see citing works
“[31], including primary carnitine deficiency (PCD) and secondary carnitine deficiency (SCD) together with metabolic pathways.”
BackgroundInfluential
The Impact of Carnitine on Dietary Fiber and Gut Bacteria Metabolism and Their Mutual Interaction in Monogastrics
Ghonimy, A., Zhang, D., Farouk, M. H., & Wang, Q. (2018) · International Journal of Molecular Sciences · doi:10.3390/ijms19041008 · Cited by 53 · see citing works
Disruption of gut integrity and permeability contributes to enteritis in a fish-parasite model: a story told from serum metabolomics
Sitjà‐Bobadilla, A., Gil‐Solsona, R., Estensoro, I., Piazzon, M. C., Martos-Sitcha, J. A., Picard‐Sánchez, A., Fuentes, J., Sancho, J. V., Calduch-Giner, J. À., Hernández, F. J., et al. (2019) · Parasites & Vectors · doi:10.1186/s13071-019-3746-7 · Cited by 52 · see citing works
Nutraceutical support in heart failure: a position paper of the International Lipid Expert Panel (ILEP)
Cicero, A. F. G., Colletti, A., von Haehling, S., Vinereanu, D., Bielecka-Dabrowa, A. M., Sahebkar, A., Toth, P. P., Reiner, Ž., Wong, N. D., Mikhailidis, D. P., et al. (2020) · Nutrition Research Reviews · doi:10.1017/s0954422420000049 · Cited by 53 · see citing works
“exerts cardioprotective effects through the reduction of oxidative stress(72) and cardiac fibrosis(73,74).”
Background
Age-related metabolic and neurodegenerative changes in SAMP8 mice
Pačesová, A., Holubová, M., Hrubá, L., Strnadová, V., Neprašová, B., Pelantová, H., Kuzma, M., Železná, B., Kuneš, J., & Maletı́nská, L. (2022) · Aging · doi:10.18632/aging.204284 · Cited by 51 · see citing works
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BackgroundInfluential
Osmoprotectants suppress the production and activity of matrix metalloproteinases induced by hyperosmolarity in primary human corneal epithelial cells.
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“Most of the L-carnitine requirement in mammals is obtained by exogenous sources from animal products; only 25% is endogenously biosynthesized from two essential amino acids, namely, lysine and methionine [12,20].”
Background
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“High in the rankings (Supplementary Table 1) were also carnitine, involved in lipid transfer across the mitochondrial membrane [24] and phenylacetylglutamine, a metabolite of glutamate, an antioxidative stress marker.”
Background
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Targeted metabolomics of nonhuman primate serum after exposure to ionizing radiation: potential tools for high-throughput biodosimetry
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“Acylcarnitines are generated as a byproduct of fatty acid oxidation [40], and accumulation of acylcarnitines in plasma is associated with secondary carnitine deficiency, impaired carnitine absorption, or poor diet [41].”
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“In the liver, carnitine deficiency has been linked to excessive lipid accumulation (13).”
Background
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Background
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Authors: Flanagan, J. L., Simmons, P. A., Vehige, J., Willcox, M. D., & Garrett, Q.
Affiliations: Brien Holden Vision Institute (AU)
Allergan (United States) (US)
Corresponding author: Flanagan, Judith L
ORCID: Flanagan, Judith L
Willcox, Mark DP
Garrett, Qian
Venue: Nutrition & Metabolism
Publisher: Springer Science and Business Media LLC
Published: 2010
Volume: 7
Issue: 1
Pages: 30
ISSN: 1743-7075 (ISSN-L)
DOI: 10.1186/1743-7075-7-30
PMC: PMC2861661
OpenAlex: W2084023408
Cited by: 611
References: 176
Citations by year: 2026: 27 · 2025: 50 · 2024: 46 · 2023: 43 · 2022: 62 · 2021: 64 · 2020: 61 · 2019: 40 · 2018: 45 · 2017: 30 · 2016: 30 · 2015: 31 · 2014: 36 · 2013: 19 · 2012: 18
FWCI: 9.03
Type: journal article
Language: en
Open access: gold
doi.org
Licence: cc-by
Locations: Nutrition & Metabolism (publishedVersion, cc-by)
PubMed Central (publishedVersion, cc-by)
PubMed (publishedVersion)
UNSWorks (University of New South Wales, Sydney, Australia) (submittedVersion, cc-by-nc-nd)
DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
Funders: Allergan
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Health Sciences › Nursing › Nutrition and Dietetics › Clinical Nutrition and Gastroenterology
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Muscle metabolism and nutrition
Keywords: L-carnitine, carnitine deficiency, carnitine transporter, fatty acid metabolism, energy production, carnitine supplementation, primary carnitine deficiency, diabetes mellitus, sepsis, cardiomyopathy, malnutrition, liver cirrhosis, aging, uremia, neuropathic pain, Alzheimer's disease, hepatic encephalopathy, dry eye, obesity, impaired glucose tolerance, cardiovascular disease

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