- 中文名称
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
- 英文名字
- Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
- 供应商
- Abbexa
- 产品货号
- abx033187
- 产品报价
- ￥询价/80ul ￥询价/400ul
- 产品说明书
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- 购买方式
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- 产品新闻
- 背景资料
- The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitiously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
- 应用类型
- WB, IHC
- 免疫原
- BCKDK
- 来源宿主
- 兔
- 反应性
- 人
- 保存建议
- 建议收到该Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody产品后，储存温度为-20°C。避免重复冻融
- 其他
- Abbexa总部位于英国剑桥的剑桥科学园,是一家专业的全球抗体供应商和蛋白质供应商,致力于为生命科学、药物开发和生物技术领域的科学家和研究者提供最佳的客户体验和高质量的产品。产品涵盖一抗、二抗、蛋白质、ELISA试剂盒、酶以及其他用于研究的试剂盒和工具。无论您是进行学术研究,药物开发,疾病研究还是其它生物学研究,Abbexa的单克隆抗体,多克隆抗体,蛋白质和生物试剂均旨在促进您的研究。如果很难找到合适的抗体来满足您的需求,Abbexa还提供了定制蛋白质,肽和抗体的生产渠道。
- 注意
-
该页面的中文产品信息的翻译，仅供参考。准确的产品信息请以厂家的英文说明书为准。下单前，请浏览说明书确认。
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