Difference between revisions of "Carnitine palmitoyltransferase I"
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|abbr=CPT | |abbr=CPT-I | ||
|description='''Carnitine palmitoyltransferase I''' (CPT | |description='''Carnitine palmitoyltransferase I''' (CPT-I, also known as carnitine acyltransferase I) is a regulatory enzyme in mitochondrial long-chain acyl-CoA uptake and further oxidation. CPT-I is associated with the mt-outer membrane mtOM and catalyses the formation of [[acylcarnitine]]s from acyl-CoA and L-carnitine. In the next step, acyl-carnitines are transported to the mitochondrial matrix via [[carnitine-acylcarnitine translocase]] in exchange for free [[carnitine]]. In the inner side of the mtIM [[carnitine palmitoyltransferase II]] converts the acyl-carnitines to carnitine and acyl-CoAs. There are three enzyme isoforms: CPT-1A (liver type), CPT-1B (muscle type), CPT-1C (brain type). Isoforms have significantly different kinetic and regulatory properties. Malonyl-CoA is an endogenous inhibitor of CPT-I. | ||
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|mitopedia topic=Enzyme | |mitopedia topic=Enzyme | ||
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Latest revision as of 16:47, 13 April 2023
- high-resolution terminology - matching measurements at high-resolution
Carnitine palmitoyltransferase I
Description
Carnitine palmitoyltransferase I (CPT-I, also known as carnitine acyltransferase I) is a regulatory enzyme in mitochondrial long-chain acyl-CoA uptake and further oxidation. CPT-I is associated with the mt-outer membrane mtOM and catalyses the formation of acylcarnitines from acyl-CoA and L-carnitine. In the next step, acyl-carnitines are transported to the mitochondrial matrix via carnitine-acylcarnitine translocase in exchange for free carnitine. In the inner side of the mtIM carnitine palmitoyltransferase II converts the acyl-carnitines to carnitine and acyl-CoAs. There are three enzyme isoforms: CPT-1A (liver type), CPT-1B (muscle type), CPT-1C (brain type). Isoforms have significantly different kinetic and regulatory properties. Malonyl-CoA is an endogenous inhibitor of CPT-I.
Abbreviation: CPT-I
MitoPedia topics:
Enzyme
Labels:
MitoPedia:FAT4BRAIN