Recombinant Human Carnitine O-palmitoyltransferase 1, liver isoform (CPT1A), Truncated

Uniprot Id

P50416

Target Species

Human

Target Name

CPT1A

Target Full Name

Carnitine O-palmitoyltransferase 1, liver isoform

Target Function

Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in hepatic triglyceride metabolism.

Target Involvement

Carnitine palmitoyltransferase 1A deficiency (CPT1AD)

Target Subcellular Location

Mitochondrion outer membrane; Multi-pass membrane protein.

Target Protein Families

Carnitine/choline acetyltransferase family

Target Tissue Specificity

Strong expression in kidney and heart, and lower in liver and skeletal muscle.

Target Synonyms

CPT1A; CPT1; Carnitine O-palmitoyltransferase 1, liver isoform; CPT1-L; Carnitine O-palmitoyltransferase I, liver isoform; CPT I; CPTI-L; Carnitine palmitoyltransferase 1A

Target Background

The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.