Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects.

Abstract excerpt

Carnitine palmitoyltransferase (CPT) deficiencies are common disorders of mitochondrial fatty acid oxidation. The CPT system is made up of two separate proteins located in the outer (CPT1) and inner (CPT2) mitochondrial membranes. While CPT2 is an ubiquitous protein, three tissue-specific CPT1 isoforms--the so-called "liver" (CPT1-A), "muscle" (CPT1B) and <<brain>> (CPT1-C) CPT1s--have been shown to exist. Amino...

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