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PDC-E2 Monoclonal Antibody

商品貨號： PLA005132

適應(yīng) 性： 人,兔

￥600元

規(guī)格：

40μL 100μL 200μL

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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

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