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Very long‐chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders
Author(s) -
Moser Hugo W.,
Moser Ann B.
Publication year - 1996
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02637066
Subject(s) - adrenoleukodystrophy , peroxisome , pathogenesis , asymptomatic , peroxisomal disorder , lipidology , clinical chemistry , medicine , fatty acid , endocrinology , bioinformatics , biology , biochemistry , receptor
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.