丙酮酸脱氢酶E2抗体
规格：1mg/1ml
英文名： PDCE2
别名： Pyruvate Dehydrogenase E2; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; anti DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide; D
分子量： 63kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PDCE2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, Guinea Pig, Cat, ,Danio rerio
细胞定位：细胞浆 线粒体
丙酮酸脱氢酶E2抗体产品介绍：background: 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 lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009] Function: The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide 丙酮酸脱氢酶E2抗体dehydrogenase (E3). Subcellular Location: Mitochondrion matrix. DISEASE: Note=Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex. Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 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丙酮酸脱氢酶E2抗体, being less prominent. Similarity: Belongs to the 2-oxoacid dehydrogenase family. Contains 2 lipoyl-binding domains. Gene ID: 1737 Database links: Entrez Gene: 512723 Cow Entrez Gene: 1737 Human Entrez Gene: 235339 Mouse Entrez Gene: 397054 Pig Entrez Gene: 81654 Rat Entrez Gene: 324201 Zebrafish Omim: 608770 Human SwissProt: P11180 Cow SwissProt: P10515 Human SwissProt: Q8BMF4 Mouse SwissProt: P08461 Rat Unigene: 335551 Human Unigene: 285076 Mouse Unigene: 471144 Mouse Unigene: 15413 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酮酸脱氢酶E2抗体产品应用：WB=1:100-500 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 新陈代谢 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid