- 中文名稱
PDHX polyclonal antibody
- 英文名字
- PDHX polyclonal antibody
- 供應商
- Bioworld
- 產品貨號
- BS8207
- 產品報價
- ¥詢價/50ul

- 產品說明書
- 點擊查看
- 購買方式
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- 產品新聞

- 背景資料
- 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.
- 應用類型
- WB, IP
- 免疫原
- Recombinant full length Human PDHX.
- 來源宿主
- Rabbit
- 反應性
- Human, Mouse, Rat
- 保存建議
- Store at 4
- 其他
- Bioworld是專一服務于生命科學研究的專業技術型企業,Bioworld品牌創立以來擁有10000多種常規產品現貨,現有8000多種現貨抗體,以及其他IVD原料,蛋白等現貨產品。在細胞信號通路、免疫學、蛋白組學上擁有顯著優勢,其抗體產品、生長因子、IVD原料等為生命科學科研工作者提供了極大的便利

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