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

Neurology

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

29 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

Bibliographical note

Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.

Keywords

Adrenoleukodystrophy
CD1
Cytokines
Hematopoietic stem cell transplantation
Immune system

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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.

KW - Adrenoleukodystrophy

KW - CD1

KW - Cytokines

KW - Hematopoietic stem cell transplantation

KW - Immune system

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Immunopathogenesis of adrenoleukodystrophy: Current understanding

Abstract

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