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折扣/货期| 货号：GTX54603 | 规格：100μl | 目录价：￥5000 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
PDHX antibody
别名:
pyruvate dehydrogenase complex component X , DLDBP , E3BP , OPDX , PDHXD , PDX1 , proX
反应种属:
Human, Mouse, Rat
宿主来源:
Rabbit
实验应用:
ICC/IF, IP, WB
同种型:
IgG
免疫原:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by affinity chromatography
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
54
产品形式:
Liquid
存储溶液:
PBS, 50% Glycerol, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
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