- 靶点:
- Pyruvate Dehydrogenase E2
- 简介:
- >>Glycolysis / Gluconeogenesis;>>Citrate cycle (TCA cycle);>>Pyruvate metabolism;>>Metabolic pathways;>>Carbon metabolism
- 基因名称:
- dlat
- Human Gene Id:
- 1737
- Human Swiss Prot No:
- P10515
- Mouse Swiss Prot No:
- Q8BMF4
- 免疫原:
- Purified recombinant human Pyruvate Dehydrogenase E2 protein fragments expressed in E.coli.
- 特异性:
- This antibody detects endogenous levels of Pyruvate Dehydrogenase E2 and does not cross-react with related proteins.
- 组成:
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源:
- Monoclonal, Mouse
- 稀释:
- wb 1:1000 icc 1:300
- 纯化工艺:
- The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
- 浓度:
- 1 mg/ml
- 储存:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称:
- 70 kDa mitochondrial autoantigen of primary biliary cirrhosis;anti DLAT;Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;Dihydrolipoamide;Dihydrolipoamide S Acetyltransferase;Dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex);Dihydrolipoamide S-Acetyltransferase;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial;DLAT;DLAT;DLTA;E2;E2 component of pyruvate dehydrogenase complex;EC 2.3.1.12;M2 antigen complex 70 kDa subunit;M2 Antigen Complex 70kD Subunit;mitochondrial;ODP2_HUMAN;PBC;PDC E2;PDC-E2;PDCE2;Pyruvate dehydrogenase complex component E2;Pyruvate dehydrogenase complex E2 subunit;S acetyltransferase component of pyruvate dehydrogenase complex.
- 实测条带:
- 69kD
- 背景:
- This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the 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 enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lact
- 功能:
- catalytic activity:Acetyl-CoA + enzyme N(6)-(dihydrolipoyl)lysine = CoA + enzyme N(6)-(S-acetyldihydrolipoyl)lysine.,cofactor:Binds 2 lipoyl cofactors covalently.,disease:Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency [MIM:245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.,disease:Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It ma
- 细胞定位:
- Mitochondrion matrix.
- 组织表达:
- Heart,Keratinocyte carcinoma,Kidney,Liver,Placenta,Testis,
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- 产品图片
- Cuproptosis-immunotherapy using PD-1 overexpressing T cell membrane-coated nanosheets efficiently treats tumor. JOURNAL OF CONTROLLED RELEASE Ling Zhang WB Mouse 1:800 4T1 cell
- Western blot detection of Pyruvate Dehydrogenase E2 in Jurkat,A549,U251,F9,Lncap and Hela cell lysates using Pyruvate Dehydrogenase E2 mouse mAb (1:1000 diluted).Predicted band size:69KDa.Observed band size:69KDa.
- Immunocytochemistry stain of Hela using Pyruvate Dehydrogenase E2 mouse mAb (1:300).
- Immunoprecipitation analysis of Hela cell lysates using Pyruvate Dehydrogenase E2 mouse mAb.