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Carnitine

From Bioblast


high-resolution terminology - matching measurements at high-resolution


Carnitine

Description

Carnitine is important factor for long-chain fatty acids transport into the mitochondrial matrix for subsequent Ξ²-oxidation. There are two enantiomers: D- and L-carnitine; however, only L-isomer is physiologically active.



MitoPedia topics: Substrate and metabolite 


Carnitine acyltransferases mediate the transport of long-chain fatty acids across the membrane by binding them to carnitine. First, long-chain fatty acids are activated by an energy-requiring step in which the fatty acid ester of CoA is formed enzymatically at the expense of ATP. The fatty acids then pass through the inner membrane and enter the mitochondria as esters of the compound carnitine. The fatty acyl group is then transferred from carnitine to intramitochondrial CoA and the resulting fatty acyl CoA is then used as a substrate by the fatty acid oxidation cycle, which occurs in the inner matrix compartment.