Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders

Hugo W. Moser; Ann B. Moser

doi:10.1007/bf02637066

Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders

Hugo W. Moser, Ann B. Moser

Kennedy Krieger Institute

Research output: Contribution to journal › Article › peer-review

32 Scopus citations

Abstract

Abnormally high levels of very long-chain fatty acids (VLCFA) are a feature in nine of the fifteen peroxisomal disorders that have been identified so far. Saturated VLCFA accumulate in X-linked adrenoleukodystrophy, appear to disrupt membrane structure, and may play a role in the pathogenesis of a brain inflammatory response. Dietary therapy initiated when patients are still asymptomatic may be of clinical benefit.

Original language	English (US)
Pages (from-to)	S-141-S-145
Journal	Lipids
Volume	31
Issue number	3 SUPPL.
DOIs	https://doi.org/10.1007/bf02637066
State	Published - Mar 1996

ASJC Scopus subject areas

Biochemistry
Organic Chemistry
Cell Biology

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10.1007/bf02637066

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abstract = "Abnormally high levels of very long-chain fatty acids (VLCFA) are a feature in nine of the fifteen peroxisomal disorders that have been identified so far. Saturated VLCFA accumulate in X-linked adrenoleukodystrophy, appear to disrupt membrane structure, and may play a role in the pathogenesis of a brain inflammatory response. Dietary therapy initiated when patients are still asymptomatic may be of clinical benefit.",

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AB - Abnormally high levels of very long-chain fatty acids (VLCFA) are a feature in nine of the fifteen peroxisomal disorders that have been identified so far. Saturated VLCFA accumulate in X-linked adrenoleukodystrophy, appear to disrupt membrane structure, and may play a role in the pathogenesis of a brain inflammatory response. Dietary therapy initiated when patients are still asymptomatic may be of clinical benefit.

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ER -

Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders

Abstract

ASJC Scopus subject areas

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