US EPA

7429224 

Journal Article 

L-carnitine and acylcarnitines: Mitochondrial biomarkers for precision medicine 

Mccann, MR; De La Rosa, MVG; Rosania, GR; Stringer, KA 

2021 

1 

Metabolites
EISSN: 2218-1989 

MDPI AG 

11 

1 

1-21 

English 

10.3390/metabo11010051 

Biomarker discovery and implementation are at the forefront of the precision medicine movement. Modern advances in the field of metabolomics afford the opportunity to readily identify new metabolite biomarkers across a wide array of disciplines. Many of the metabolites are derived from or directly reflective of mitochondrial metabolism. L-carnitine and acylcarnitines are established mitochondrial biomarkers used to screen neonates for a series of genetic disorders affecting fatty acid oxidation, known as the inborn errors of metabolism. However, L-carnitine and acylcarnitines are not routinely measured beyond this screening, despite the growing evidence that shows their clinical utility outside of these disorders. Measurements of the carnitine pool have been used to identify the disease and prognosticate mortality among disorders such as diabetes, sepsis, cancer, and heart failure, as well as identify subjects experiencing adverse drug reactions from various medications like valproic acid, clofazimine, zidovudine, cisplatin, propofol, and cyclosporine. The aim of this review is to collect and interpret the literature evidence supporting the clinical biomarker application of L-carnitine and acylcarnitines. Further study of these metabolites could ultimately provide mechanistic insights that guide therapeutic decisions and elucidate new pharmacologic targets. © 2021 by the authors. Licensee MDPI, Basel, Switzerland. 

Acyl-carnitine; Metabolic flexibility; Metabolism; Metabolomics; Mitochondria; Pharmacometabolomics; acylcarnitine; adenosine triphosphate; biological marker; branched chain amino acid; carbamate kinase; carbamoyl phosphate synthase; carnitine; carnitine palmitoyltransferase; cisplatin; clofazimine; cyclosporine; fatty acid; hemoglobin A1c; long chain fatty acid; mitochondrial biomarker; propofol; RNA directed DNA polymerase; unclassified drug; valproic acid; zidovudine; adverse drug reaction; bioenergy; C2C12 cell line; chemical structure; diabetes mellitus; diet supplementation; glucose metabolism; glycolysis; human; metabolite; mitochondrial respiration; mitochondrion; mortality; nonalcoholic fatty liver; nonhuman; personalized medicine; respiratory chain; Review; sepsis; septic shock; upregulation