Recombinant Human Carnitine O-palmitoyltransferase 2, mitochondrial (CPT2), Truncated

Uniprot Id

P23786

Target Species

Human

Target Name

CPT2

Target Full Name

Carnitine O-palmitoyltransferase 2, mitochondrial

Target Function

Involved in the intramitochondrial synthesis of acylcarnitines from accumulated acyl-CoA metabolites. Reconverts acylcarnitines back into the respective acyl-CoA esters that can then undergo beta-oxidation, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Active with medium (C8-C12) and long-chain (C14-C18) acyl-CoA esters.

Target Involvement

Carnitine palmitoyltransferase 2 deficiency, myopathic, stress-induced (CPT2D); Carnitine palmitoyltransferase 2 deficiency, infantile (CPT2DI); Carnitine palmitoyltransferase 2 deficiency, lethal neonatal (CPT2DLN); Encephalopathy, acute, infection-induced, 4 (IIAE4)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Carnitine/choline acetyltransferase family

Target Research Area

Cancer

Target Synonyms

Carnitine O palmitoyltransferase 2; Carnitine O palmitoyltransferase 2 mitochondrial; Carnitine O-palmitoyltransferase 2; Carnitine palmitoyltransferase 2; Carnitine palmitoyltransferase II; CPT 1; CPT 2; CPT II; CPT1; CPT2; CPT2_HUMAN; CPTASE; CPTII; IIAE4; mitochondrial

Target Background

The protein encoded by this gene is a nuclear protein which is transported to the mitochondrial inner membrane. Together with carnitine palmitoyltransferase I, the encoded protein oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders.