Oxidation of very-long-chain fatty acids in rat brain: cerotic acid is β-oxidized exclusively in rat brain peroxisomes

HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes

“…ABCD1 mutation/deletion and/or the metabolites on expression of other transporters in X-ALD fi broblasts. Earlier studies from our laboratory ( 7,(71)(72)(73)(74) and others ( 75 ) have reported that VLCFAs are preferentially ␤ -oxidized in peroxisomes, whereas oxidation of palmitic acid occurs largely in mitochondria. Treatment with SAHA increased both the lignoceric acid (C24:0) and palmitic acid (C16:0) ␤ -oxidation activities in X-ALD fi broblasts.…”

Lovastatin and sodium phenylacetate normalize the levels of very long chain fatty acids in skin fibroblasts of X‐ adrenoleukodystrophy

“…A number of laboratories, including ours, showed that β‐oxidation of VLCFA is a peroxisomal function [3–7]. The deficient activity of lignoceroyl‐CoA ligase as compared to the normal oxidation of lignoceroyl‐CoA in purified peroxisomes isolated from fibroblasts of X‐ALD suggested that the abnormality in the oxidation of VLCFA may be due to deficient activity of lignoceroyl‐CoA ligase required for the activation of lignoceric acid to lignoceroyl‐CoA [4, 6].…”

Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes

“…Previously, studies from our laboratory [44, 59–61] and others [62] have documented that VLCFA metabolism is restricted to peroxisomes while LCFA β-oxidation is attributable largely to mitochondria. Treatment with CAPE, at doses that upregulated ALDRP expression, significantly increased both VLCFA and LCFA β-oxidation in control and X-ALD fibroblasts.…”