L-Carnitine is required to shuttle long-chain fatty acids across the inner mitochondrial membrane via the CPT1/carnitine-acylcarnitine-translocase/CPT2 system, the rate-limiting entry point for mitoch...
L-Carnitine is required to shuttle long-chain fatty acids across the inner mitochondrial membrane via the CPT1/carnitine-acylcarnitine-translocase/CPT2 system, the rate-limiting entry point for mitochondrial beta-oxidation. Genetic OCTN2 transporter defects that impair carnitine uptake cause primary carnitine deficiency, underscoring the pathway's centrality to fatty-acid-based cellular energy metabolism.
Endocrinology · 2020
Reviews CPT1A as the rate-limiting hepatic enzyme converting acyl-CoA to acyl-carnitine for mitochondrial fatty-acid oxidation entry, and its genetic/nutritional regulation.
View Source →Biochimica et Biophysica Acta · 2016
Details how carnitine transfers long-chain fatty acids across the inner mitochondrial membrane for beta-oxidation, and how OCTN2 transporter defects cause primary carnitine deficiency.
View Source →Current Drug Metabolism · 2011
Reviews L-carnitine's role transporting fatty-acid chains into the mitochondrial matrix, noting promising but still limited evidence for applications in heart failure and weight management.
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