[CV(%) = SD/mean x100].Storage Stability: The loss activity rate of this kit is less than 8% within the expiration date under appropriate storage condition.Cat.No: MBS
异丁酰辅酶A脱氢酶缺乏(Deficiency of isobutyryl-CoA dehydrogenase)基因检测就是线粒体基因检测吗? 是的,异丁酰辅酶A脱氢酶缺乏是一种线粒体基因突变导致的遗传疾病,因此基因检测可以通过检测线粒体基因来确定是否存在该疾病。异丁酰辅酶A脱氢酶缺乏(Deficiency of isobutyryl-CoA dehydrogenase)的有效治好性治疗会...
REFERENCESBattaileKP,etal.Structuresofisobutyryl-CoAdehydrogenaseandenzyme-productcomplex:comparisonwithisovaleryl-andshort-chainacyl-CoAdehydrogenases.JBiolChem.2004Apr16;279(16):16526-34.McePdfHeightCaution:Producthasnotbeenfullyvalidatedformedicalapplications.Forresearchuseonly.Tel:400-820-3792FaxE-mail:tech...
英文别名: ACAD8 (ARC42, IBD, Isobutyryl-CoA Dehydrogenase, Mitochondrial, Activator-recruited Cofactor 42kD Component, Acyl-CoA Dehydrogenase Family Member 8, ACAD-8, FLJ22590) (MaxLight 650)中文名: 中文别名: CBNumber: CB04959519 分子式: 分子量: 0 ...
BProblem statement:/B Human Isobutyryl-CoA Dehydrogenase (IBD) is member of the Acyl-CoA Dehydrogenases family. It is involved in Val metabolism. In this study we modified the purification method of the IBD and further spectroscopic characterization was conducted. BApproach:/B Using DTT in the ...
208–210 More recently, acyl-CoA dehydrogenase 9 (ACAD-9) was discovered.211 This enzyme is most active with unsaturated long-chain acyl-CoAs.212 Substrates whose acyl chain length is too long for a particular ACAD are excluded sterically by the length of the hydrophobic tunnel the alkyl ...
Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA. Isobutyryl-CoA is a substrate for isobutyryl-CoA dehydrogenase (IBDH) in the catabolism of valine and an intermediate in the synthesis of isobutyryl-L-carnitine , which accumulates in IBDH deficiency. ...
Isobutyryl-L-carnitine is a natural 4-carbon acylcarnitine that is involved in fatty acid oxidation and organic acid metabolism.1Elevated levels of isobutyryl-L-carnitine are associated with isobutyryl-CoA dehydrogenase deficiency.2,3 1.Turer, A.T., Stevens, R.D., Bain, J.R., et al.Metabol...
5A,B), such as aconitate hydratase, malate dehydrogenase, isocitrate dehydrogenase, transketolase, succinate–CoA ligase, succinate dehydrogenase, pyruvate dehydrogenase complex, citrate synthase, glyceraldehyde-3-phosphate dehydrogenase, phosphoglycerate kinase, pyruvate kinase, fructose-bisphosphate aldolase, 6...
Herbert Irving Comprehen- sive Cancer Center, College of Physicians and Surgeons, Columbia University, 1130 Nicholas Avenue, New York, NY 10032, USA Short-chain fatty acids and their corresponding acyl-CoAs sit at the crossroads of metabolic pathways and play important roles in diverse cellular pro...