Peroxisomal Pathways, their Role in Neurodegenerative Disorders and Therapeutic Strategies

摘要: X-linked adrenoleukodystrophy (X-ALD), the most frequent peroxisomal disease, is due to a mutation in ABCD1 gene encoding for protein important transport of acyl chains into peroxisomes further oxidation. This leads defect this transporter and an accumulation very long chain fatty acids (VLCFA), particular 26:0, plasma tissues, including brain. The high concentration saturated VLCFA alters structure functions cell membranes, toxic effects are attributable induction oxidative stress inflammation (also associated with autoimmune mechanisms). Dietary intervention therapy have provided encouraging results, albeit sometimes conflicting findings different clinical studies. Elucidation molecular mechanisms X-ALD should aid developing new strategies, e.g. synergistic approach acting on causes pharmacological therapies X-ALD.