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PDH-E1α Monoclonal Antibody

商品貨號(hào)： PLA005133

適應(yīng) 性： 人,小鼠,大鼠,牛,狗,豬

￥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： 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

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