Immunopathogenesis of adrenoleukodystrophy: Current understanding

Michelle P. Hudspeth, Gerald V Raymond

Research output: Contribution to journalReview articlepeer-review

24 Scopus citations

Abstract

Adrenoleukodystrophy is a neurometabolic disease with a decreased ability to degrade very long chain fatty acids (VLCFA) and significant phenotypic variation. Unlike most neurometabolic diseases, the success of hematopoietic stem cell transplantation (HSCT) is based on acquiring a new immune system rather than enzyme replacement. VLCFA accumulation appears necessary but not sufficient for pathogenesis. Evidence for the involvement of different components of the immune system in the pathogenesis of the cerebral lesions (cellular, cytokines, humoral, and complement) is reviewed, along with both HSCT and non-HSCT immunologic approaches to treatment and future directions.

Original languageEnglish (US)
Pages (from-to)5-12
Number of pages8
JournalJournal of Neuroimmunology
Volume182
Issue number1-2
DOIs
StatePublished - Jan 1 2007

Keywords

  • Adrenoleukodystrophy
  • CD1
  • Cytokines
  • Hematopoietic stem cell transplantation
  • Immune system

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