Pyruvate Dehydrogenase Complex, Component X (PDHX) (Middle Region) Peptide
PDHX
适用: 人
宿主: 合成
BP, WB
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- 抗原 See all PDHX products
- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
- 蛋白结构域
- Middle Region
- 宿主
- 人
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资源
- 合成
- 应用范围
- Blocking Peptide (BP), Western Blotting (WB)
- 序列
- FRLSPAARNI LEKHSLDASQ GTATGPRGIF TKEDALKLVQ LKQTGKITES
- 产品特性
- This is a synthetic peptide designed for use in combination with anti- PDHX Antibody. It may block above mentioned antibody from binding to its target protein in western blot and/or immunohistochecmistry under proper experimental settings. There is no guarantee for its use in other applications.
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- 应用备注
- Optimal working dilution should be determined by the investigator.
- 限制
- 仅限研究用
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- 状态
- Lyophilized
- 溶解方式
- Add 100 μL of sterile PBS. Final peptide concentration is 1 mg/mL in PBS.
- 储存条件
- -20 °C
- 储存方法
- For longer periods of storage, store at -20°C. Avoid repeat freeze-thaw cycles.
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- 抗原
- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
- 背景
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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.
Alias Symbols: E3BP, OPDX, PDX1, proX, DLDBP
Protein Size: 501 - 基因ID
- 8050
- NCBI登录号
- NM_001135024, NP_001128496
- UniProt
- O00330
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