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首頁 > 抗體 > 一抗 > 其它 > PDC-E2 Monoclonal Antibody
PDC-E2 Monoclonal Antibody
商品貨號： PLA005132
適 應(yīng) 性： 人,兔
WB
￥600元
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MSDS
說明書
商品描述
  • 發(fā)貨日期： 7
  • 基因名稱： DLAT
  • 蛋白名稱： Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
  • Human_gene_id： 1737
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1737
  • Human_swiss_prot_no： P10515
  • Human_swiss_link： http://www.uniprot.org/uniprotkb/P10515/entry
  • Mouse_swiss_prot_no： Q8BMF4
  • Mouse_swiss_link： http://www.uniprot.org/uniprot/Q8BMF4
  • Rat_swiss_prot_no： P08461
  • Rat_swiss_link： http://www.uniprot.org/uniprot/O54941P08461
  • 特異性： PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來源： Monoclonal, Mouse
  • 稀釋： WB 1:1000 - 1:2000. Not yet tested in other applications.
  • 純化工藝： Affinity purification
  • 濃度： 1 mg/ml
  • 儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： DLAT; DLTA; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; mitochondrial; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruva
  • 信號通路： Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Pyruvate metabolism;
  • 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 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.,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).,sequence caution:Contaminating sequence. Sequence of unknown origin in the N-terminal part.,similarity:Belongs to the 2-oxoacid dehydrogenase family.,similarity:Contains 1 lipoyl-binding domain.,similarity:Contains 2 lipoyl-binding domains.,subunit:20 to 30 alpha(2)-beta(2) tetramers of E1 + 6 homodimers of E3 + 60 copies of E2.,
  • 相關(guān)產(chǎn)品： YM1328,YM1071,RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達： Heart,Keratinocyte carcinoma,Kidney,Liver,Placenta,Testis,
  • tag： hot
  • 科研貨號： PLA005132
PDC-E2 Monoclonal Antibody
Catalog No PLA005132
Product information
  • 發(fā)貨日期： 7
  • 基因名稱： DLAT
  • 蛋白名稱： Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
  • Human_gene_id： 1737
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1737
  • Human_swiss_prot_no： P10515
  • Human_swiss_link： http://www.uniprot.org/uniprotkb/P10515/entry
  • Mouse_swiss_prot_no： Q8BMF4
  • Mouse_swiss_link： http://www.uniprot.org/uniprot/Q8BMF4
  • Rat_swiss_prot_no： P08461
  • Rat_swiss_link： http://www.uniprot.org/uniprot/O54941P08461
  • 特異性： PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來源： Monoclonal, Mouse
  • 稀釋： WB 1:1000 - 1:2000. Not yet tested in other applications.
  • 純化工藝： Affinity purification
  • 濃度： 1 mg/ml
  • 儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： DLAT; DLTA; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; mitochondrial; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruva
  • 信號通路： Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Pyruvate metabolism;
  • 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 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.,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).,sequence caution:Contaminating sequence. Sequence of unknown origin in the N-terminal part.,similarity:Belongs to the 2-oxoacid dehydrogenase family.,similarity:Contains 1 lipoyl-binding domain.,similarity:Contains 2 lipoyl-binding domains.,subunit:20 to 30 alpha(2)-beta(2) tetramers of E1 + 6 homodimers of E3 + 60 copies of E2.,
  • 相關(guān)產(chǎn)品： YM1328,YM1071,RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達： Heart,Keratinocyte carcinoma,Kidney,Liver,Placenta,Testis,
  • tag： hot
  • 科研貨號： PLA005132
    • Hunan UPT Biotechnology Co.,Ltd
    Website：www.jx3dscan.com Servive hotline :4006916686
    E-mail：service@uptbio.com
    Address：
    Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.
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