Immunopathogenesis of adrenoleukodystrophy: Current understanding

Michelle P. Hudspeth, Gerald V. Raymond

Research output: Contribution to journalReview articlepeer-review

29 Scopus citations


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
Issue number1-2
StatePublished - Jan 2007

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Neurology
  • Clinical Neurology


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