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Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs?
Author(s) -
Djouadi F.,
Aubey F.,
Schlemmer D.,
Gobin S.,
Laforet P.,
Wanders R. J. A.,
Strauss A. W.,
Bonnefont J. P.,
Bastin J.
Publication year - 2006
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-006-0266-3
Subject(s) - beta oxidation , pharmacology , medicine , fatty acid , chemistry , biochemistry
Summary Exposure to fibrates leads to normalization of fatty acid oxidation (FAO) in fibroblasts from patients with myopathic forms of CPT2 deficiency or VLCAD deficiency. Correction of FAO is related to a drug‐induced increase of residual enzyme activity, and this could provide a new treatment strategy for these disorders.