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Role of carnitine in disease
Open accessAbstract
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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“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]
In the citing paper
“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]
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“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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“Furthermore, we calculated the ratio of carnitine C 2:0 and carnitine which reflects the extent of the betaoxidation [32].”
MethodVidot, 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
“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.”
BackgroundChe, 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 52
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Effect of L‐carnitine on the synthesis of nitric oxide in RAW 264·7 murine macrophage cell line
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“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
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“[31], including primary carnitine deficiency (PCD) and secondary carnitine deficiency (SCD) together with metabolic pathways.”
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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 55
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“exerts cardioprotective effects through the reduction of oxidative stress(72) and cardiac fibrosis(73,74).”
BackgroundAge-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 55
“The blood plasma concentration of leptin was measured using an ELISA kit (Millipore, St. Charles, MI, USA), free fatty acids (FFAs) were analyzed using a colorimetric assay (Roche Mannheim, Germany), insulin was analyzed using RIA kits (Millipore, St. Charles, MI, USA), and triacylglycerols (TAGs) and cholesterol were…”
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Production of L-carnitine-enriched edible filamentous fungal biomass through submerged cultivation
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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].”
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Role of Zinc and Copper in Erythropoiesis in Patients on Hemodialysis
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Potential Therapeutic Role of Carnitine and Acetylcarnitine in Neurological Disorders
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Serum metabolomics analysis of patients with polycystic ovary syndrome by mass spectrometry
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Ascorbic acid metabolites are involved in intraocular pressure control in the general population
Hysi, P. G., Khawaja, A. P., Menni, C., Tamraz, B., Wareham, N. J., Khaw, K., Foster, P. J., Benet, L. Z., Spector, T. D., & Hammond, C. J. (2018) · Redox Biology · doi:10.1016/j.redox.2018.10.004 · Cited by 44
“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.”
BackgroundA Distinctive Human Metabolomics Alteration Associated with Osteopenic and Osteoporotic Patients
Aleidi, S. M., Alnehmi, E. A., Alshaker, M., Masood, A., Benabdelkamel, H., Al‐Ansari, M. M., & Rahman, A. M. A. (2021) · Metabolites · doi:10.3390/metabo11090628 · Cited by 47
Diet, nutraceuticals and the tear film
Jalbert, I. (2013) · Experimental Eye Research · doi:10.1016/j.exer.2013.08.016 · Cited by 46
“Nutritional supplements of Lcarnitine may be of benefit for a range of conditions including uremia, diabetes, cardiovascular disease, obesity and Alzheimer’s disease but have not been tested specifically for their effectiveness in modulating tear film health (Flanagan et al., 2010).”
BackgroundPannkuk, E. L., Laiakis, E. C., Authier, S., Wong, K. K.-W., & Fornace, A. J. (2016) · RSC Advances · doi:10.1039/c6ra07757a · Cited by 46
Childhood growth and neurocognition are associated with distinct sets of metabolites
Moreau, G. B., Ramakrishnan, G., Cook, H. L., Fox, T. E., Nayak, U., Ma, J. Z., Colgate, E. R., Kirkpatrick, B. D., Haque, R., & Petri, W. A. (2019) · EBioMedicine · doi:10.1016/j.ebiom.2019.05.043 · Cited by 48
“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).”
BackgroundEffects of L-carnitine supplementation on lipid profiles in patients with coronary artery disease
Lee, B., Lin, J.-S., Lin, Y., & Lin, P. (2016) · Lipids in Health and Disease · doi:10.1186/s12944-016-0277-5 · Cited by 48
“LC may carry long-chain fatty acids across the inner mitochondrial membrane for βoxidation and adenosine triphosphate (ATP) production [1, 2].”
BackgroundHeart dysfunction induced by choline-deficiency in adult rats: The protective role of l-carnitine
Strilakou, A. A., Lazaris, A. C., Perelas, A. I., Mourouzis, I. S., Douzis, I. C., Karkalousos, P. L., Stylianaki, A. T., Pantos, C. I., & Liapi, C. A. (2013) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2013.03.025 · Cited by 36
Metadata
- 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
- Venue
- Nutrition & Metabolism
- Published
- 2010
- Volume
- 7
- Issue
- 1
- Pages
- 30
- ISSN
- 1743-7075 (ISSN-L)
- 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
- Health Sciences › Nursing › Nutrition and Dietetics › 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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