Significance of l‐carnitine for human health

  title={Significance of l‐carnitine for human health},
  author={Mar{\'i}a M. Adeva-Andany and Isabel Calvo‐Castro and Carlos Fern{\'a}ndez-Fern{\'a}ndez and Crist{\'o}bal Donapetry-Garc{\'i}a and Ana Mar{\'i}a Pedre-Pi{\~n}eiro},
  journal={IUBMB Life},
Carnitine acyltransferases catalyze the reversible transfer of acyl groups from acyl‐coenzyme A esters to l‐carnitine, forming acyl‐carnitine esters that may be transported across cell membranes. l‐Carnitine is a wáter‐soluble compound that humans may obtain both by food ingestion and endogenous synthesis from trimethyl‐lysine. Most l‐carnitine is intracellular, being present predominantly in liver, skeletal muscle, heart and kidney. The organic cation transporter‐2 facilitates l‐carnitine… 

Carnitine in Human Muscle Bioenergetics: Can Carnitine Supplementation Improve Physical Exercise?

The role of l-carnitine in muscle energetics and the main causes that led to conflicting data on the use of l/s carnitine as a supplement are described.

The Importance of the Fatty Acid Transporter L-Carnitine in Non-Alcoholic Fatty Liver Disease (NAFLD)

Studies that show reduced intrahepatic fat and reduced liver enzymes after L-carnitine supplementation suggest that L- carnitines might be a promising supplement to improve or delay the progression of NAFLD.

Role of Carnitine in Non-alcoholic Fatty Liver Disease and Other Related Diseases: An Update

Based on the “multiple hit” hypothesis, carnitine inhibits β-oxidation, improves mitochondrial dysfunction, and reduces insulin resistance to ameliorate NAFLD.

The prospect of using L-carnitine for modification of drinking mineral waters in order to increase their metabolic potential

Based on the results of independent scientific studies, it is unambiguous to conclude that there is both a direct and a more complex effect of mineral waters on impaired metabolism caused by magnesium and calcium deficiency in this group of patients.

Coenzyme Q 10 and L-Carnitine Disturbances in Children with Mitochondrial Diseases

A lower absolute level of coenzyme Q10 and tendency toward a more pronounced decrease in the Q10/cholesterol ratio in older patients (6–16 years) were shown and the free carnitine blood level was within the normal range.

Logistic role of carnitine shuttle system on radiation-induced L-carnitine and acylcarnitines alteration.

Evidence indicates that that there is a logistic role of carnitine shuttle system on radiation-induced L-carnitine and acylc Carnitines alteration.

Carnitine promotes recovery from oxidative stress and extends lifespan in C. elegans

It is shown that L-carnitine improves recovery from oxidative stress and extends lifespan in C. elegans, and an important role is suggested in oxidative stress recovery that might be important for healthy aging in humans.

Exometabolome profiling reveals activation of the carnitine buffering pathway in fed‐batch cultures of CHO cells co‐fed with glucose and lactic acid

A comprehensive semi‐quantitative and time‐dependent analysis of the exometabolome revealed several carnitine derivatives as biomarkers of the simultaneous activation of TCA anaplerotic pathways as well as a carbon‐buffering pathway that would supply more pyruvate through oxidative phosphorylation.



Carnitine palmitoyltransferase 2 and carnitine/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines

  • S. ViolanteL. Ijlst S. Houten
  • Biology
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology
  • 2013
CPT2 and CACT are crucial for mitochondrial acylcarnitine formation and export to the extracellular fluids in mFAOD, and results obtained using pharmacological inhibition of CPT2 in fibroblasts from MCAD‐deficient individuals show this.

The mitochondrial carnitine/acylcarnitine carrier: function, structure and physiopathology.

OCTN3 is a mammalian peroxisomal membrane carnitine transporter.

This work demonstrates expression and colocalization of mOctn3, the intermediate-affinity carnitine transporter, and catalase in murine liver peroxisomes by TEM using immunogold labelled anti-m Octn3 and anti-catalase antibodies, and supports the localization of hOCTN3 to theperoxisomal membrane.

Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient.

Pharmacokinetics of L-Carnitine

The renal clearance of L-carnitine increases after exogenous administration, approaching GFR after high intravenous doses, and many forms of secondary carnitine deficiency, including some drug-induced disorders, arise from impaired renal tubular re absorption.

A novel brain-expressed protein related to carnitine palmitoyltransferase I.

A novel CPT1 family member whose mRNA is present predominantly in brain and testis is described, whose protein sequence contains all the residues known to be important for both carnitine acyltransferase activity and malonyl-CoA binding in other family members.

Disorders of carnitine biosynthesis and transport.