- 中文名称
PDHX polyclonal antibody
- 英文名字
- PDHX polyclonal antibody
- 供应商
- Bioworld
- 产品货号
- BS8207
- 产品报价
- ￥询价/50ul
- 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 pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. E3BP (E3-binding protein), also known as PDHX (Pyruvate dehydrogenase protein X component) and Lipoyl-containing pyruvate dehydrogenase complex component X, is a 501 amino acid mitochondrial protein that is required for anchoring E3 to the E2 core of the PDH complex, an event that is essential for a functional PDH complex. Defects in the gene encoding E3BP result in pyruvate dehydrogenase E3-binding protein deficiency, which is similar to PDH deficiency and Leigh syndrome in clinical presentation. Symptoms of E3BP deficiency can include lactic acidosis, delayed development, seizures, diplegia, cerebellar ataxia, optic atrophy, facial dysmorphism and episodic weakness.
- 应用类型
- WB, IP
- 免疫原
- Recombinant full length Human PDHX.
- 来源宿主
- Rabbit
- 反应性
- Human, Mouse, Rat
- 保存建议
- Store at 4
- 其他
- Bioworld是专一服务于生命科学研究的专业技术型企业，Bioworld品牌创立以来拥有10000多种常规产品现货，现有8000多种现货抗体，以及其他IVD原料，蛋白等现货产品。在细胞信号通路、免疫学、蛋白组学上拥有显著优势，其抗体产品、生长因子、IVD原料等为生命科学科研工作者提供了极大的便利
- 注意
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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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