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Rabbit Anti-PDCE2/BF488 Conjugated antibody (bs-19695R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-19695R-BF488
英文名稱 Rabbit Anti-PDCE2/BF488 Conjugated antibody
中文名稱 BF488標記的丙酮酸脫氫酶E2抗體
別    名 Pyruvate Dehydrogenase E2; 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; 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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細胞生物  神經(jīng)生物學(xué)  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, Guinea Pig, Cat, ,Danio rerio)
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 63kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PDCE2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 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 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, being less prominent.

Similarity:
Belongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.

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.
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