Immunopathogenesis of adrenoleukodystrophy: Current understanding

Michelle P. Hudspeth; Gerald V. Raymond

doi:10.1016/j.jneuroim.2006.10.009

Immunopathogenesis of adrenoleukodystrophy: Current understanding

Michelle P. Hudspeth, Gerald V. Raymond

School of Medicine

Research output: Contribution to journal › Review article › peer-review

25 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 language	English (US)
Pages (from-to)	5-12
Number of pages	8
Journal	Journal of Neuroimmunology
Volume	182
Issue number	1-2
DOIs	https://doi.org/10.1016/j.jneuroim.2006.10.009
State	Published - Jan 2007

Keywords

Adrenoleukodystrophy
CD1
Cytokines
Hematopoietic stem cell transplantation
Immune system

ASJC Scopus subject areas

Immunology and Allergy
Immunology
Neurology
Clinical Neurology

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10.1016/j.jneuroim.2006.10.009

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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.",

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AB - 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.

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KW - Cytokines

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Immunopathogenesis of adrenoleukodystrophy: Current understanding

Abstract

Keywords

ASJC Scopus subject areas

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