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首頁 > 抗體 > 一抗 > 其它 > PDH-E1α Monoclonal Antibody
PDH-E1α Monoclonal Antibody
商品貨號(hào)： PLA005133
適 應(yīng) 性： 人,小鼠,大鼠,牛,狗,豬
WB IF
￥600元
規(guī)格：
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MSDS
說明書
商品描述
  • 發(fā)貨日期： 7
  • 基因名稱： PDHA1 ODPA
  • 蛋白名稱： Pyruvate dehydrogenase E1 component subunit alpha somatic form mitochondrial
  • Human_gene_id： 5160
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5160
  • Human_swiss_prot_no： P08559
  • Human_swiss_link： http://www.uniprot.org/uniprotkb/P08559/entry
  • Mouse_gene_id： 18597
  • Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18597
  • Mouse_swiss_prot_no： P35486
  • Mouse_swiss_link： http://www.uniprot.org/uniprot/P35486
  • Rat_swiss_prot_no： P26284
  • Rat_swiss_link： http://www.uniprot.org/uniprot/P26284
  • 特異性： PDH-E1α Monoclonal Antibody detects endogenous levels of PDH-E1α 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. IF 1:100 - 1:500. Not yet tested in other applications.
  • 純化工藝： Affinity purification
  • 濃度： 1 mg/ml
  • 儲(chǔ)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： PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I
  • 信號(hào)通路： Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Valine; leucine and isoleucine biosynthesis;Pyruvate metabolism;Butanoate metabolism;
  • 功能： catalytic activity:Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).,cofactor:Thiamine pyrophosphate.,disease:Defects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).,disease:Defects in PDHA1 are the cause of X-linked Leigh syndrome (LS) [MIM:308930]. LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.,enzyme regulation:E1 activity is regulated by phosphorylation (inactivation) and dephosphorylation (activation) of the alpha subunit.,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).,subunit:Tetramer of 2 alpha and 2 beta subunits.,tissue specificity:Ubiquitous.,
  • 相關(guān)產(chǎn)品： YT3641,YN0062,YM1234,YM1072
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA005133
PDH-E1α Monoclonal Antibody
Catalog No PLA005133
Product information
  • 發(fā)貨日期： 7
  • 基因名稱： PDHA1 ODPA
  • 蛋白名稱： Pyruvate dehydrogenase E1 component subunit alpha somatic form mitochondrial
  • Human_gene_id： 5160
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5160
  • Human_swiss_prot_no： P08559
  • Human_swiss_link： http://www.uniprot.org/uniprotkb/P08559/entry
  • Mouse_gene_id： 18597
  • Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18597
  • Mouse_swiss_prot_no： P35486
  • Mouse_swiss_link： http://www.uniprot.org/uniprot/P35486
  • Rat_swiss_prot_no： P26284
  • Rat_swiss_link： http://www.uniprot.org/uniprot/P26284
  • 特異性： PDH-E1α Monoclonal Antibody detects endogenous levels of PDH-E1α 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. IF 1:100 - 1:500. Not yet tested in other applications.
  • 純化工藝： Affinity purification
  • 濃度： 1 mg/ml
  • 儲(chǔ)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： PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I
  • 信號(hào)通路： Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Valine; leucine and isoleucine biosynthesis;Pyruvate metabolism;Butanoate metabolism;
  • 功能： catalytic activity:Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).,cofactor:Thiamine pyrophosphate.,disease:Defects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).,disease:Defects in PDHA1 are the cause of X-linked Leigh syndrome (LS) [MIM:308930]. LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.,enzyme regulation:E1 activity is regulated by phosphorylation (inactivation) and dephosphorylation (activation) of the alpha subunit.,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).,subunit:Tetramer of 2 alpha and 2 beta subunits.,tissue specificity:Ubiquitous.,
  • 相關(guān)產(chǎn)品： YT3641,YN0062,YM1234,YM1072
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA005133
    • 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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