PDHX 抗体 (Middle Region)
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- 抗原 See all PDHX 抗体
- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
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抗原表位
- Middle Region
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适用
- 人
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This PDHX antibody is un-conjugated
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应用范围
- Western Blotting (WB)
- 序列
- NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP
- 纯化方法
- Affinity purified
- 免疫原
- The immunogen is a synthetic peptide directed towards the middle region of human PDHX
- Top Product
- Discover our top product PDHX Primary Antibody
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anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 220-468) antibody
PDHX 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 222-471) antibodyPDHX 适用: 小鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 1-300) antibodyPDHX 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 1-501) antibodyPDHX 适用: 人 WB, IF 宿主: 小鼠 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (C-Term) antibodyPDHX 适用: 人 WB, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 1-501) antibodyPDHX 适用: 人 WB, IP 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 1-109) antibodyPDHX 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (Thr11) antibodyPDHX 适用: 人 WB, IF, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (C-Term) antibodyVerified PDHX 适用: 人 WB, ELISA 宿主: 山羊 Polyclonal unconjugated
anti-Pyruvate Dehydrogenase Complex, Component X (PDHX) (AA 1-300) antibodyPDHX 适用: 人 WB, IP 宿主: 兔 Polyclonal unconjugated
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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
- Liquid
- 缓冲液
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
- 储存液
- Sodium azide
- 注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
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- 抗原
- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
- 别名
- PDHX (PDHX 产品)
- 别名
- DLDBP antibody, E3BP antibody, OPDX antibody, PDX1 antibody, proX antibody, AI481367 antibody, E3bp antibody, Pdx1 antibody, Ac1164 antibody, zgc:66110 antibody, zgc:85977 antibody, MGC86218 antibody, PDHX antibody, DDBDRAFT_0168431 antibody, DDBDRAFT_0230192 antibody, DDB_0168431 antibody, DDB_0230192 antibody, pyruvate dehydrogenase complex component X antibody, pyruvate dehydrogenase complex, component X antibody, pyruvate dehydrogenase complex component X L homeolog antibody, E3-binding protein antibody, PDHX antibody, Pdhx antibody, pdhx antibody, pdhx.L antibody, pdhX antibody
- 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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