Reactome: A Curated Pathway Database

Import of palmitoyl-CoA into the mitochondrial matrix (R-HSA-200425)

Species 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).

Locations in the PathwayBrowser
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