Cerebral inflammation in X-linked adrenoleukodystrophy

Martina C. McGuinness, Kirby D. Smith

Research output: Contribution to journalArticle

Abstract

X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disease that affects approximately 1 in 25 000 males. It is characterized by elevated levels of saturated very long chain fatty acids (VLCFA), i.e., >C22:0, particularly in ganglioside and cholesterol ester fractions of brain white matter and adrenal cortex. Failure of peroxisomal very long chain fatty acyl-CcA synthetase (VLCS) to activate these VLCFA prevents their degradation by peroxisomal β-oxidation. X-ALD maps to Xq2833 and the gene encodes a peroxisomal membrane protein39, 40, 65 and not the gene for VLCS. The two most common forms of X-ALD are the cerebral (CER) form, with an inflammatory demyelinating reaction that resembles multiple sclerosis (MS), and adrenomyeloneuropathy (AMN), which involves the spinal cord and in which the inflammatory reaction is mild or absent. Investigations into the nature of the cerebral inflammatory demyelinating reaction in X-ALD will be the subject of this review.

Original languageEnglish (US)
Pages (from-to)281-287
Number of pages7
JournalArchivum Immunologiae et Therapiae Experimentalis
Volume47
Issue number5
StatePublished - Dec 1 1999

Keywords

  • Cytokines
  • Demyelination
  • Human leukocyte antigens
  • Inflammation
  • X-linked adrenoleukodystrophy

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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