中文名称:2-Methylbutyryl-CoA 英文名称:2-Methylbutyryl-CoA CAS No.:6712-02-3 分子式:C26H44N7O17P3S分子量:851.6505 国内供应商 现货直采 了解现货直采业务 上海楷森生物科技有限公司 产品编号品牌纯度包装库存价格 FM113CHEMSOONBR/95%以上10mg现货/定制面议 ...
2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism. Pediatr Res. 2000;47:830-3.Gibson KM, Burlingame G, Hogema B, Jakobs C, Schutgens RBH, Millington D, Roe CR, Roe DS, Sweetman L, Steiner RD, Linck L, Pohowalla P, Sacks M, Kiss D...
products from lysed cells, using ferricenium as an electron acceptor. The enzyme activity in the patient's fibroblasts when 2-methylbutyryl-CoA was used as substrate was shown to be decreased to 10% of controls (control, 0.157 nmol/min/mg protein; patient, 0.016 nmol/min/mg protein [±10...
2-Methylbutyrate2-MethylvalerateAscarisNematodeMitochondriaA branched-chain acyl-CoA transferase activity which transfers coenzyme A from either 2-methylbutyryl or 2-methylvaleryl-CoA to succinate is present in the muscle mitochondria from the intestinal nematode, Ascaris suum. Its physiological function ...
Case Study for 2-Methylbutyryl CoA Dehydrogenase DeficiencyChapman, MScotia, Nova
3-Hydroxy-2-methylbutyryl-CoA dehydrogenaseNo Abstract available for this chapter.doi:10.1007/978-3-642-57756-7_28Professor Dr. Dietmar SchomburgGBF — Gesellschaft für Biotechnologische Forschung mbHDr. Dörte StephanGBF — Gesellschaft für Biotechnologische Forschung mbHSpringer Berlin Heidelberg...
, et al. ( 2000 ) Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism . Am J Hum Genet...