Difference between revisions of "Carnitine palmitoyltransferase I"

Revision as of 11:23, 22 January 2023

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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 IA (liver type), CPT IB (muscle type), CPT IC (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

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 {{MitoPedia
-|abbr=CPT 1
+|abbr=CPT I
-|description='''Carnitine palmitoyltransferase I''' (CPT 1, also known as carnitine acyltransferase I) is a regulatory enzyme in mitochondrial long-chain acyl-CoA uptake and further oxidation. CPT 1 is associated with the outer mt-membrane 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 2]] 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 1.
+|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 IA (liver type), CPT IB (muscle type), CPT IC (brain type). Isoforms have significantly different kinetic and regulatory properties. Malonyl-CoA is an endogenous inhibitor of CPT I.
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 {{MitoPedia topics
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