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PDHA1/2 rabbit pAb
商品貨號(hào)： PLA021452
適 應(yīng) 性： 人,小鼠,大鼠
WB
￥600元
規(guī)格：
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MSDS
說(shuō)明書
商品描述
  • 發(fā)貨日期： 14
  • 基因名稱： PDHA1 PHE1A
  • 蛋白名稱： PDHA1/2
  • 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： https://www.uniprot.org/uniprotkb/P08559/entry
  • Mouse_gene_id： 18597
  • Mouse_gene_link： https://www.uniprot.org/uniprot/18597
  • Mouse_swiss_prot_no： P35486
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P35486
  • Rat_swiss_prot_no： P26284
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/P26284
  • 特異性： This antibody detects endogenous levels of PDHA1/2 at Human, Mouse,Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1:500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (EC 1.2.4.1) (PDHE1-A type I)
  • 分子量： 43kD
  • 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： RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA021452
PDHA1/2 rabbit pAb
Catalog No PLA021452
Product information
  • 發(fā)貨日期： 14
  • 基因名稱： PDHA1 PHE1A
  • 蛋白名稱： PDHA1/2
  • 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： https://www.uniprot.org/uniprotkb/P08559/entry
  • Mouse_gene_id： 18597
  • Mouse_gene_link： https://www.uniprot.org/uniprot/18597
  • Mouse_swiss_prot_no： P35486
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P35486
  • Rat_swiss_prot_no： P26284
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/P26284
  • 特異性： This antibody detects endogenous levels of PDHA1/2 at Human, Mouse,Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1:500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 其他名稱： Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (EC 1.2.4.1) (PDHE1-A type I)
  • 分子量： 43kD
  • 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： RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Mitochondrion matrix.
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA021452
    • Hunan UPT Biotechnology Co.,Ltd
    Website：www.zjgzfxx.com Servive hotline :4006916686
    E-mail：service@uptbio.com
    Address：
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