Reactome: A Curated Pathway Database

Import of palmitoyl-CoA into the mitochondrial matrix (R-HSA-200425) [Homo sapiens]


The mitochondrial carnitine system catalyzes the transport of long-chain fatty acids into the mitochondrial matrix where they undergo beta oxidation. This transport system consists of the malonyl-CoA sensitive carnitine palmitoyltransferase I (CPT-I) localized in the mitochondrial outer membrane, the carnitine:acylcarnitine translocase, an integral inner membrane protein, and carnitine palmitoyltransferase II localized on the matrix side of the inner membrane. (Kerner and Hoppel, 2000).

Additional Information
Compartment mitochondrion , cytosol
GO Biological Process carnitine shuttle (0006853)
Literature References
pubMedId Title Journal Year
11257506 Molecular enzymology of carnitine transfer and transport Biochim Biophys Acta 2001