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货号： bs-5003R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5003R-Cy7
- 英文名称
- Anti-PDHX/Cy7
- 中文名称
- Cy7标记的丙酮酸脱氢酶复合物X蛋白抗体
- 别 名
- Dihydrolipoamide dehydrogenase binding protein of pyruvate dehydrogenase complex; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex; DLDBP; E3 binding protein; E3-binding protein; E3BP; Lipoyl containing pyruvate dehydrogenase complex component X; Lipoyl-containing pyruvate dehydrogenase complex component X; mitochondrial; ODPX_HUMAN; OPDX; PDHX; PDX 1; PDX1; Pro X; ProX; Pyruvate dehydrogenase complex component X; Pyruvate dehydrogenase complex lipoyl containing component X; Pyruvate dehydrogenase complex, E3 binding protein subunit; Pyruvate dehydrogenase protein X component; Pyruvate dehydrogenase protein X component mitochondrial.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 48kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PDHX
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
PDHX is component X of the pyruvate dehydrogenase (PDH) complex.It is required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex. PDHX is expressed in the mithochondrion.
Function:
Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
Subunit:
Part of the inner core of the multimeric pyruvate dehydrogenase complex that is composed of about 48 DLAT and 12 PDHX molecules. This core binds multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3).
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349]: A metabolic disorder characterized by decreased activity of the pyruvate dehydrogenase complex without observable reduction in the activities of enzymes E1, E2, or E3. Clinical features include hypotonia and psychomotor retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the 2-oxoacid dehydrogenase family.
Contains 1 lipoyl-binding domain.
Database links:
UniProtKB/Swiss-Prot: O00330.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.