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description
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)

external resources
NCBI:1270014
REACTOME:R-HSA-200425
PUBMED:11257506

genes
SLC25A20 , CPT1A , CPT1B , CPT2 , PPARD , PRKAA2 , PRKAB2 , RXRA , THRSP , PRKAG2 , MID1IP1 ,