Long-chain carnitine acyltransferases are a family of enzymes found in
mitochondria, peroxisomes, and endoplasmic reticulum that catalyze th
e exchange of carnitine for coenzyme A in the fatty acyl-CoA. Conversi
on of the fatty acyl-CoA to fatty acylcarnitine renders the fatty acid
more permeable to the various cellular membranes. The mitochondrial c
arnitine palmitoyltransferases are considered important in the regulat
ion of mitochondrial beta-oxidation of long-chain fatty acids. However
, palmitoylcarnitine produced by peroxisomal carnitine octanoyltransfe
rase or by microsomal carnitine palmitoyltransferase is not different
from that produced by the mitochondrial enzyme. Therefore, for there t
o be control of fatty acid oxidation by the long-chain carnitine acylt
ransferases, there would have to be some mechanism to coordinately reg
ulate these varied enzymes. The first system of regulation involves in
hibition by malonyl-CoA, an intermediate in the synthesis of fatty aci
ds. Malonyl-CoA inhibits long-chain carnitine acyltransferase activity
by all three enzymes at similar concentrations in the physiological r
ange. In addition, the mitochondrial and peroxisomal enzymes are known
to be regulated at the level of mRNA transcription by a number of sha
red factors. Although the microsomal enzyme is less well studied, ther
e does, indeed, appear to be a pattern of coordinate regulation for th
is system.