Catalyzes the reversible transfer of acyl groups from carnitine to coenzyme A (CoA) and regulates the acyl-CoA/CoA ratio. Also plays a crucial role in the transport of fatty acids for beta-oxidation . Responsible for the synthesis of short- and branched-chain acylcarnitines . Active towards some branched-chain amino acid oxidation pathway (BCAAO) intermediates . Trans-2-enoyl-CoAs and 2-methylacyl-CoAs are poor substrates .
Endoplasmic reticulum . Peroxisome . Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .; [Isoform 1]: Mitochondrion .; [Isoform 2]: Peroxisome .
Mostly in skeletal muscle, less in heart, liver and pancreas, only weakly detectable in brain, placenta, lung and kidney.
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