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Difference between revisions of "Carnitine palmitoyltransferase I"

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{{MitoPedia
|abbr=CPT I
|abbr=CPT-I
|description=CPT I is a regulatory step in mitochondrial long-chain acylCoA uptake and further oxidation. CPT I is associated with outer mitochondrial membrane and catalyses the formation of acylcarnitines from acylCoA and L-carnitine. Β 
|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.
There are 3 enzyme isoforms: CPT IA (liver type), CPT IB (muscle type), CPT IC (brain type). Isoforms have significantly different kinetic and regulatory properties. MalonylCoA is endogenous inhibitor of CPT I.
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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 


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MitoPedia:FAT4BRAIN