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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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“Carnitine binds fatty acids, generating various acylcarnitines with different chain lengths (Flanagan et al., 2010).”
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“An anti–inflammatory effect by protease-induced catecholamine release is also plausible, though requires further investigations 15 .”
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Hong, Y., Li, L.-H., Kuo, T.-H., Lee, Y., & Hsu, C. (2025) · Journal of the American Society for Mass Spectrometry · doi:10.1021/jasms.5c00009 · Cited by 2
Beyond newborn screening: the role of reverse cascade testing in familial disease detection
Gaviglio, A., Petritis, K., Tagi, V. M., Vasco, A., Mauri, A., Zuccotti, G., Verduci, E., Cereda, C., & Ferraro, S. (2025) · Critical Reviews in Clinical Laboratory Sciences · doi:10.1080/10408363.2025.2527288 · Cited by 2
“For these cases RCS is recommended, as the mother’s status may put her at risk for future adverse events (i.e. cardiovascular and musculoskeletal disorders, hepatic involvement, and neurodegeneration) [65,66].”
Loomba, R. S., Villarreal, E. G., Patel, R. D., Udarbe, S., Dorsey, V., Nelson-McMillan, K. L., & Flores, S. (2020) · Congenital Heart Disease · doi:10.32604/chd.2020.012927 · Cited by 2
Rhabdomyolysis as a late complication of bariatric surgery
Rigney, L., El‐Haddad, C., Cappelen‐Smith, C., Pamphlett, R., Gotis‐Graham, I., & Cordato, D. J. (2016) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2016.03.027 · Cited by 2
Nutritional Supplementation in Ehlers-Danlos Syndrome
Castori, M., Bruschini, M., & Blundo, C. (2014) · Elsevier eBooks · doi:10.1016/b978-0-12-411462-3.00017-5 · Cited by 2
Type II Diabetes, Peripheral Neuropathy, and Gout
Ettinger, S. E. (2016) · Elsevier eBooks · doi:10.1016/b978-0-12-803013-4.00004-1 · Cited by 2
Carnitine Profile Changes in Pediatric Hematopoietic Stem Cell Transplant: New Role for Carnitine?
Law, S. F., Fenton, T. R., Snyder, F. F., Fung, E., & Lewis, V. A. (2020) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0000000000001723 · Cited by 2
Merante, F. (2020) · Biomedical Journal of Scientific & Technical Research · doi:10.26717/bjstr.2020.24.004087 · Cited by 2
“Also included in the synergistic blend was ascorbic acid, which confers cellular and mitochondrial antioxidant properties [25, 26] and supports the biosynthesis of carnitine [27,28], an essential shuttling molecule for redistribution of carbon skeletons within the cells.”
BackgroundShahouzehi, B., Fallah, H., & Masoumi-Ardakani, Y. I. (2020) · The Ukrainian Biochemical Journal · doi:10.15407/ubj92.05.033 · Cited by 2
Fasting hypoglycaemia secondary to carnitine deficiency: a late consequence of gastric bypass
Chen, X., Kimura, B. H., Nagelberg, J., & McCowen, K. C. (2021) · BMJ Case Reports · doi:10.1136/bcr-2021-241703 · Cited by 2
Evaluation of carnitine levels in dried blood spot samples in children with autism spectrum disorder
Aslan, O., Kardaş, B., Özbek, M. N., & Ercan, B. (2021) · Turkish Journal of Biochemistry · doi:10.1515/tjb-2020-0420 · Cited by 3
“Although carnitine plays a major role in free fatty acid metabolism, carnitine increases the use of carbohydrates [11].”
BackgroundGhanem, I., Nashy, B. N. E., Sarhan, S., & Shaker, A. (2022) · Zagazig University Medical Journal · doi:10.21608/zumj.2022.156980.2622 · Cited by 2
Animal-Based Bioactive Components: Zoochemicals: A Comprehensive Review
Sindhav, R. G., Hazra, T., & Mudgil, D. (2022) · Springer eBooks · doi:10.1007/978-981-19-2366-1_3 · Cited by 2
Metabolic and Metabolomic Effects of Metformin in Murine Model of Pulmonary Adenoma Formation
Elton, A. C., Cedarstrom, V., Quraishi, A., Wuertz, B. R. K., Murray, K. J., Markowski, T. W., Seabloom, D. E., & Ondrey, F. G. (2023) · Nutrition and Cancer · doi:10.1080/01635581.2023.2165692 · Cited by 2
“Some of these metabolites, particularly 2-aminoadipic acid and the carnitine derivatives, are associated with lysine degradation though were not included in the pathway hits, which may represent a limitation of the pathway analysis softwares (45, 46).”
BackgroundClofazimine-Mediated, Age-Related Changes in Skeletal Muscle Mitochondrial Metabolites
Diaz‐Espinosa, J., Stringer, K. A., & Rosania, G. R. (2023) · Metabolites · doi:10.3390/metabo13050671 · Cited by 2
Association between plasma L-carnitine levels and mitochondrial DNA copy number
Li, M., Yang, K., Vivo, I. D., Eliassen, A. H., Qureshi, A. A., Nan, H., & Han, J. (2023) · BMC Molecular and Cell Biology · doi:10.1186/s12860-023-00496-z · Cited by 2
“Furthermore, carnitine aids in enhancing glucose consumption and maintains its energy-producing capacity despite changes in osmolytic pressure [14, 16–18].”
BackgroundLipid profiles of rats fed with diets supplemented with vitamins niacin and pyridoxine
Lameiro, M. D. G. S., Helbig, E., Zavareze, E. D. R., de Moura, F. A., Tavares, R. A., Vargas, C. G., Borges, L. R., Elias, M. C., & Dias, Á. R. G. (2012) · DOAJ (DOAJ: Directory of Open Access Journals) · Cited by 2
Hogan, P., Plourde, R., Fortier, M., Brindamour, D., Verdant, C. L., Demers-Marcil, S., & Dupuis, S. (2018) · Journal of Pharmacy Practice · doi:10.1177/0897190018782012 · Cited by 2
“There are 2 different types of carnitine deficiency, primary and secondary.(2) Primary deficiency is caused by genetic deficiencies in carnitine transporters, whereas secondary deficiency can have multiple causes such as increased elimination, malnutrition, malabsorption, and drug induction.”
BackgroundInfluentialXu, D. Z., & You, G. (2017) · American Journal of Physiology-Cell Physiology · doi:10.1152/ajpcell.00093.2017 · Cited by 2
“L-Carnitine, the active form, plays an important role in oxidizing fatty acids, transporting long chain fatty acids across mitochondrial membrane, and modulating intracellular coenzyme A homeostasis (3).”
BackgroundA Case of Atypical Systemic Primary Carnitine Deficiency in Saudi Arabia
Alghamdi, A. E., Almalki, H., Shawli, A. M., Waggass, R., & Hakami, F. (2018) · Pediatric Reports · doi:10.4081/pr.2018.7705 · Cited by 1
L-carnitine Improves Atherosclerotic Plaques in Hyperlipidemic Animal Model (Hyperlipidemic Rabbits)
Zarei, L., Farhad, N., Abbasi, A., & Haddadi, S. N. (2022) · Egyptian Journal of Veterinary Science · doi:10.21608/ejvs.2022.133398.1338 · Cited by 1
“About 75% of LC in the body is obtained from diet [10].”
BackgroundL-carnitine attenuates acoustic startle reflex dysfunction in adult male rats exposed to mancozeb
Dehghani, A., Pourjafari, F., Koohkan, F., Haghpanh, T., Pourjafari, F., Sheibani, V., & Afarinesh, M. R. (2023) · Toxicology and Industrial Health · doi:10.1177/07482337231151739 · Cited by 1
“Because L-carnitine exerts its effects through the mechanisms involved in the control of destructive oxidant agents (Simmons et al., 2010), the purpose of this investigation was to examine the possible effects of an optimal dose of L-carnitine on locomotor activity and the acoustic startle response…”
BackgroundInfluentialFamilial dilated cardiomyopathy in a child: a case report
Ismail, A., Khreis, D., Assaad, A., & Majdalani, M. N. (2024) · BMC Pediatrics · doi:10.1186/s12887-024-04614-4 · Cited by 1
“Cardioprotective effects of L-carnitine also include inhibition of cardiac fibrosis, nitric oxide generation, and interstitial myocardial remodeling [30].”
BackgroundMikami-Saito, Y., Maekawa, M., Watanabe, M., Hosaka, S., Takahashi, K., Totsune, E., Arai‐Ichinoi, N., Kikuchi, A., Kure, S., Katagiri, H., et al. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms26072962 · Cited by 1
“…with type 2 diabetes, particularly those with diabetic complications, face an increased likelihood of carnitine deficiency and may develop additional risk factors, such as hemodialysis-requiring renal failure and nonalcoholic fatty liver disease, further exacerbating the condition [34–36].”
Akan, G., Balcı, M. C., Tuncel, G., Karaca, M., Kazan, H. H., Ozketen, A. C., Özgen, Ö., Gökçay, G., & Atalar, F. (2025) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-025-03775-4 · Cited by 1
“In contrast, SCD is more common, milder and often associated with liver or kidney disease or medications like valproic acid [4, 8–9].”
InfluentialQian, D., & Cui, Q. (2025) · Applied Water Science · doi:10.1007/s13201-025-02495-1 · Cited by 1
Puente-Fernández, J., Larumbe‐Zabala, E., Roberts, J., & Naclerio, F. (2025) · Journal of the International Society of Sports Nutrition · doi:10.1080/15502783.2025.2519515 · Cited by 1
“It may promote vasodilation and improve oxygen supply to the working muscles [16].”
Li, Y., Xiao, H., Liu, L., Jiang, F., & Yu, E. (2025) · Ecotoxicology and Environmental Safety · doi:10.1016/j.ecoenv.2025.119352 · Cited by 2
Longitudinal characterization of fecal lipids in dairy heifers from birth to first calving
Ghaffari, M. H., Sadri, H., Hemmert, K. J., Ostendorf, C. S., Schuchardt, S., Koch, C., & Sauerwein, H. (2025) · Journal of Dairy Science · doi:10.3168/jds.2025-27816 · Cited by 1
Risk factors for carnitine deficiency in critically ill adults: A descriptive cross‐sectional study
Gordon, J., Brody, R. A., Samavat, H., Hamdan, M., Colonna, A. L., Young, J., & Byham-Gray, L. D. (2026) · Journal of Parenteral and Enteral Nutrition · doi:10.1002/jpen.70044 · Cited by 1
“Critical illness places patients at risk for L ‐ carnitine deficiency (hereafter referred to as carnitine) because of protein catabolism 1 and possible related treatments including dialysis, 2 parenteral nutrition, 3 and medications such as valproic acid.”
Beyond the Usual: Novel Therapeutic Roles for Established Antidotes
Koppen, A., Dijkman, M. A., Visser, C. C., & de Lange, D. W. (2026) · Therapeutic Drug Monitoring · doi:10.1097/ftd.0000000000001433 · Cited by 1
Therapeutic potential of L-carnitine in coronary artery disease: a systematic review
Werida, R. H., & Okda, S. M. (2026) · Inflammopharmacology · doi:10.1007/s10787-025-02048-7 · Cited by 1
“This is particularly relevant because the cardiac muscle has limited capacity to synthesize LC (Flanagan et al. 2010) .”
Wu, W., Zhou, M., Chen, J., Wang, J., Hong, Q., Li, Y., Wang, Y., Zhang, P., Huang, Y., Zhang, Z., et al. (2026) · Med · doi:10.1016/j.medj.2026.101033 · Cited by 1
Hathiramani, N. R., Jerrett, A. E., & Spinelli, J. B. (2026) · Trends in Endocrinology and Metabolism · doi:10.1016/j.tem.2026.03.007 · Cited by 1
Furusho, A., Mizuno, H., Nakayama, Y., Sugiyama, E., Kojima, K., & Todoroki, K. (2026) · Journal of Pharmaceutical and Biomedical Analysis Open · doi:10.1016/j.jpbao.2026.100111 · Cited by 1
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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