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丙酮酸脫氫酶復合物X蛋白抗體
  • 產(chǎn)品貨號：
    BN41032R
  • 中文名稱：
    丙酮酸脫氫酶復合物X蛋白抗體
  • 英文名稱：
    Rabbit anti-PDHX Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

    備注

  • BN41032R-100ul

    100ul

    ￥2360.00

    交叉反應：Mouse,Rat(predicted:Human,Dog) 推薦應用：WB,ELISA

  • BN41032R-200ul

    200ul

    ￥3490.00

    交叉反應：Mouse,Rat(predicted:Human,Dog) 推薦應用：WB,ELISA

產(chǎn)品描述

英文名稱PDHX
中文名稱丙酮酸脫氫酶復合物X蛋白抗體
別    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.  
研究領(lǐng)域腫瘤  細胞生物  免疫學  信號轉(zhuǎn)導  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應Mouse, Rat,  (predicted: Human, Dog, )
產(chǎn)品應用WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量48kDa
細胞定位細胞漿 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human PDHX:231-330/501 
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹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.

SWISS:
O00330

Gene ID:
8050

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.