The enzymes for the b-oxidation
oflong- to mediumchain fatty acids (C-18 to C-12) are located close to the inner mitochondrial membrane, they are: very-longchain acyl-CoA dehydrogenase, long-chain enoyl-CoA hydratase, long-chain L 3-hydroxyacyl-CoA dehydrogenase and long-chain 3-ketoacyl-CoA thiolase.
Novel fatty acid B-oxidation
enzymes in rat liver mitochondria I.
The altered b-oxidation
is followed by the accumulation of representative acylcarnitines depending on the type of enzymatic deficiency in the pathway (1).
The main function of carnitine is to shuttle activated long-chain fatty acids [fatty acylcoenzyme A (CoA)] from the cytosol into the mitochondrial matrix for b-oxidation
, and to remove short-chain, medium-chain and long chain fatty acids, that accumulate as a result of normal and abnormal metabolism (37).
Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial ayl-CoA dehydrogenase gen product, is a rate-limiting enzyme in long-chain fatty acid b-oxidation