Affiliations 

  • 1 School of Medicine, Fu Jen Catholic University, New Taipei City, Taiwan ROC
Pediatr Neurol, 2013 Sep;49(3):185-90.
PMID: 23835273 DOI: 10.1016/j.pediatrneurol.2013.04.021

Abstract

X-linked adrenoleukodystrophy is caused by a defective peroxisomal membrane transporter, ABCD1, responsible for transporting very-long-chain fatty acid substrate into peroxisomes for degradation. The main biochemical defect, which is also one of the major diagnostic hallmarks, of X-linked adrenoleukodystrophy is the accumulation of saturated very-long-chain fatty acids in all tissues and body fluids.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.