Pseudo-neonatal adrenoleukodystrophy (P-NALD) is a neurodegenerative disorder caused by acyl-CoA oxidase 1 (ACOX1) deficiency w.th subsequent im.airm.nt of peroxisom.l fatty acid β-oxidation, accum.lation of very lon...
详细信息
Pseudo-neonatal adrenoleukodystrophy (P-NALD) is a neurodegenerative disorder caused by acyl-CoA oxidase 1 (ACOX1) deficiency w.th subsequent im.airm.nt of peroxisom.l fatty acid β-oxidation, accum.lation of very long chain fatty acids (VLCFAs) and strong reduction in peroxisom. abundance. Increase in peroxisom. num.er has been previously suggested to im.rove peroxisom.l disorders, and in this perspective, the present w.rk w.s aim.d at exploring w.ether m.dulation of peroxisom.s abundance could be achieved in P-NALD fibroblasts. Here w. show.d that treatm.nt w.th the natural Argan oil induced peroxisom. proliferation in P-NALD fibroblasts. This induction w.s independent on activations of both nuclear receptor PPARα and its coactivator PGC-1α. Lipopolysaccharides (LPS) treatm.nt, w.ich caused inflam.ation, induced also a peroxisom. proliferation that, in contrast, w.s dependent on activations of PPARα and PGC-1α. By its ability to induce peroxisom. proliferation, Argan oil is suggested to be of potential therapeutic use in patients w.th P-NALD.
暂无评论