Carnitine Palmitoyltransferase I (CPTI)

Function

CPT 1 is an enzyme that is present at the cytosol side of the outer mitochondrial membrane. It has the function of transferring the acyl group on to the carnitine group from the acyl CoA group, allowing transportation of acyl group into the mitochondria through the carinitine carrier.

Regulation

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Figure 1. The location of CPT1 on the outermembrane of the mitochondrion adapted from Van de Leij (2000)

The inhibitor of CPT 1 is malonyl CoA, which are products of a reaction catalyzed by acetyl CoA carboxylase. The different isoforms of CPT1 react differently to the malonyl CoA (ie. the muscle isoform more sensitive to malonyl CoA than liver isoform). [1]

Video explanation of transport



Pathway 2
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  1. ^ Van der Leij, F.R. (2000). Mol. Genet. Metab. 71, 139