Fluorometric assay of acyl-CoA dehydrogenases in normal and mutant human fibroblasts.
A fluorimetric, ETF-linked procedure to determine activities of acyl-CoA dehydrogenase in cultured human fibroblasts is described. The assay readily distinguishes between cell lines deficient in medium-chain acyl-CoA dehydrogenase, long-chain acyl-CoA dehydrogenase, isovaleryl-CoA dehydrogenase, and controls, and may allow for the diagnosis of heterozygous carriers of these disorders. The method has been made feasible with the development of rapid and efficient procedures to isolate ETF, and offers several advantages over procedures that are currently employed.
D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000032:analysis*; D044927:Electron-Transferring Flavoproteins; D005347:Fibroblasts / Q000201:enzymology*; D005420:Flavoproteins / Q000378:metabolism; D005470:Fluorometry; D006801:Humans; D007506:Iron-Sulfur Proteins; D008956:Models, Chemical; D009097:Multienzyme Complexes; D009154:Mutation; D010088:Oxidoreductases / Q000378:metabolism; D000587:Oxidoreductases Acting on CH-NH Group Donors; D013053:Spectrophotometry; D014451:Ubiquinone / Q000378:metabolism
D044927:Electron-Transferring Flavoproteins; D005420:Flavoproteins; D007506:Iron-Sulfur Proteins; D009097:Multienzyme Complexes; D014451:Ubiquinone; D010088:Oxidoreductases; D044942:Acyl-CoA Dehydrogenase, Long-Chain; D000587:Oxidoreductases Acting on CH-NH Group Donors; C023120:electron-transferring-flavoprotein dehydrogenase