TITLE:
Modulation of peroxisomes abundance by argan oil and lipopolysaccharides in acyl-CoA oxidase 1-deficient fibroblasts
AUTHORS:
Riad El Kebbaj, Soufiane El Kamouni, Hammam I. El Hajj, Pierre Andreoletti, Joseph Gresti, Norbert Latruffe, M’Hammed Saïd El Kebbaj, Joseph Vamecq, Gérard Lizard, Boubker Nasser, Mustapha Cherkaoui-Malki
KEYWORDS:
Acyl-CoA Oxidase 1; Argan Oil; LPS; PGC-1a; Peroxisome Proliferation; P-NALD; PPARa
JOURNAL NAME:
Health,
Vol.5 No.1,
January
11,
2013
ABSTRACT:
Pseudo-neonatal adrenoleukodystrophy (P-NALD) is a neurodegenerative disorder caused by acyl-CoA oxidase 1 (ACOX1) deficiency with subsequent impairment of peroxisomal fatty acid β-oxidation, accumulation of very long chain fatty acids (VLCFAs) and strong reduction in peroxisome abundance. Increase in peroxisome number has been previously suggested to improve peroxisomal disorders, and in this perspective, the present work was aimed at exploring whether modulation of peroxisomes abundance could be achieved in P-NALD fibroblasts. Here we showed that treatment with the natural Argan oil induced peroxisome proliferation in P-NALD fibroblasts. This induction was independent on activations of both nuclear receptor PPARα and its coactivator PGC-1α. Lipopolysaccharides (LPS) treatment, which caused inflammation, induced also a peroxisome proliferation that, in contrast, was dependent on activations of PPARα and PGC-1α. By its ability to induce peroxisome proliferation, Argan oil is suggested to be of potential therapeutic use in patients with P-NALD.