In light of their growing importance, we have undertaken an extensive review of acylcarnitines and provided a detailed description of their identity, nomenclature, classification, biochemistry, pathophysiology, supplementary use, potential drug targets, and clinical trials.

Acetyl-L-carnitine, ALCAR or ALC, is an acetylated form of L - carnitine. It is naturally produced by the human body, and it is available as a dietary supplement. Acetylcarnitine is broken down in the blood by plasma esterases to carnitine which is used by the body to transport fatty acids into the mitochondria for breakdown and energy production.

Many metabolites are closely tied to components of mitochondrial function, but herein L-carnitine (LC) and the acylcarnitines (ACs) will be discussed for their untapped potential to serve as biomarkers for illness and drug response, including adverse drug reactions (ADRs).

The acyl groups are conjugated with carnitine to form acylcarnitines, which can be measured by tandem mass spectrometry. Diagnostic results are usually characterized by a pattern of significantly elevated acylcarnitine species compared to normal and disease controls.

Acylcarnitines play an essential role in regulating the balance of intracellular sugar and lipid metabolism. They serve as carriers to transport activated long-chain fatty acids into mitochondria for β-oxidation as a major source of energy for cell activities. The liver is the most important organ for endogenous carnitine synthesis and metabolism.

Oct 19, 2020 · Acylcarnitine analysis is a useful test for identifying patients with inborn errors of mitochondrial fatty acid β-oxidation and certain organic acidemias. Plasma is routinely used in the...

Acylcarnitines are esters arising from conjugation of a broad range of acyl chains to the hydroxyl group of L-carnitine (L-ß-hydroxy-4-N -trimethylaminobutyric acid). Carnitine is well known to play a prominent role in transport of long-chain fatty acids from cytosol to …

Acylcarnitines are esters arising from the conjugation of fatty acids (i.e., acyl groups) with L-carnitine. They are widely used and produced in cellular energy metabolism pathways.

Sep 1, 2012 · Given the importance of L-carnitine as well as acylcarnitines in maintaining normal mitochondrial function, this review seeks to examine previous research associated with the homeostasis and pharmacokinetics of L-carnitine and its esters, and highlight potential areas of future research.

Recent data indicate new, multifactorial roles for acylcarnitines in neuroprotection. Brain acylcarnitines can function in synthesizing lipids, altering and stabilizing membrane composition, modulating genes and proteins, improving mitochondrial function, increasing antioxidant activity, and enhancing cholinergic neurotransmission.