caa a22 4500 46904506X CHVBK 20180323132822.0 cr unu---uuuuu 170328e19920901xx s 000 0 eng 10.1007/BF01800014 doi (NATIONALLICENCE)springer-10.1007/BF01800014 Measurement of acyl-CoA dehydrogenase activity in cultured skin fibroblasts and blood platelets [Elektronische Daten] [R. Taylor, S. Jackson, M. Pourfarzam, K. Bartlett, D. Turnbull] 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. SSIEM and Kluwer Academic Publishers, 1992 Taylor R. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut Jackson S. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut Pourfarzam M. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut Bartlett K. Department of Child Health, The Medical School, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut Turnbull D. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 727-732 0141-8955 15:5<727 1992 15 10545 https://doi.org/10.1007/BF01800014 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800014 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Taylor R. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut NATIONALLICENCE 700 1- Jackson S. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut NATIONALLICENCE 700 1- Pourfarzam M. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut NATIONALLICENCE 700 1- Bartlett K. Department of Child Health, The Medical School, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut NATIONALLICENCE 700 1- Turnbull D. Division of Clinical Neuroscience, University of Newcastle upon Tyne, Framlington Place, NE2 4HH, Newcastle upon Tyne, UK aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 727-732 0141-8955 15:5<727 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer