Carnitine metabolism

Stable Identifier
R-HSA-200425
DOI
Type
Pathway
Species
Homo sapiens
Compartment
Synonyms
Import of palmitoyl-CoA into the mitochondrial matrix
ReviewStatus
5/5
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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).
Literature References
PubMed ID Title Journal Year
11257506 Molecular enzymology of carnitine transfer and transport

Gandour, RD, Ramsay, RR, van der Leij, FR

Biochim Biophys Acta 2001
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