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Measurement of acyl‐CoA dehydrogenase activity in cultured skin fibroblasts and blood platelets
Author(s) -
Taylor R. W.,
Jackson S.,
Pourfarzam M.,
Bartlett K.,
Turnbull D. M.
Publication year - 1992
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/bf01800014
Subject(s) - dehydrogenase , biochemistry , lactate dehydrogenase , enzyme , chemistry , enzyme assay , platelet , electron acceptor , biology , immunology
Summary The measurement of acyl‐CoA dehydrogenase activity is an essential part of the investigation of patients with suspected defects of fatty acid oxidation, and recently the organometallic oxidant ferricenium hexafluorophosphate has been introduced as an electron acceptor for these assays. However, we show that when medium‐chain acyl‐CoA dehydrogenase activity was measured in cultured skin fibroblasts and platelets from patients with proven defects of this enzyme, there was considerable residual enzyme activity when this electron acceptor was used. The ferricenium assay is not as specific as the anaerobic ETF‐linked assay in the biochemical diagnosis of medium‐chain acyl‐CoA dehydrogenase deficiency in fibroblasts, and therefore is of limited clinical applicability in its present form.