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Merck
CN

AV48136

Sigma-Aldrich

Anti-PDHA1 (AB1) antibody produced in rabbit

IgG fraction of antiserum

别名:

Anti-PDHA, Anti-PDHCE1A, Anti-PHE1A, Anti-Pyruvate dehydrogenase (lipoamide) α 1

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关于此项目

UNSPSC代码:
12352203
NACRES:
NA.41
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生物来源

rabbit

质量水平

偶联物

unconjugated

抗体形式

IgG fraction of antiserum

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous solution

分子量

40 kDa

种属反应性

human, mouse, guinea pig, rabbit, dog, rat

浓度

0.5 mg - 1 mg/mL

技术

western blot: suitable

NCBI登记号

UniProt登记号

运输

wet ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... PDHA1(5160)

一般描述

PDHA1 codes for the α1 subunit of the pyruvate dehydrogenase complex and modulates the activity of the complex. PDHA1 mutations have been implicated in pyruvate dehydrogenase deficiency.
Rabbit Anti-PDHA1 antibody recognizes bovine, chicken, pig, human, mouse, rat, and canine PDHA1.

免疫原

Synthetic peptide directed towards the N terminal region of human PDHA1

应用

Rabbit Anti-PDHA1 antibody is suitable for western blot applications at a concentration of 5μg/ml.

生化/生理作用

The pyruvate dehydrogenase complex is a nuclear-encoded mitochondrial matrix multienzyme complex that provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The PDH complex is composed of multiple copies of 3 enzymes: E1 (PDHA1); dihydrolipoyl transacetylase (DLAT); and dihydrolipoyl dehydrogenase (DLD). The E1 enzyme is a heterotetramer of 2 alpha and 2 beta subunits. The E1-alpha subunit contains the E1 active site and plays a key role in the function of the PDH complex.The pyruvate dehydrogenase complex is a nuclear-encoded mitochondrial matrix multienzyme complex that provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The PDH complex is composed of multiple copies of 3 enzymes: E1 (PDHA1); dihydrolipoyl transacetylase (DLAT; MIM 608770) (E2; EC 2.3.1.12); and dihydrolipoyl dehydrogenase (DLD; MIM 238331) (E3; EC 1.8.1.4). The E1 enzyme is a heterotetramer of 2 alpha and 2 beta subunits. The E1-alpha subunit contains the E1 active site and plays a key role in the function of the PDH complex (Brown et al., 1994 [PubMed 7853374]).[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

外形

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

其他说明

Synthetic peptide located within the following region: MRKMLAAVSRVLSGASQKPASRVLVASRNFANDATFEIKKCDLHRLEEGP

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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分析证书(COA)

Lot/Batch Number

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E Ostergaard et al.
Journal of inherited metabolic disease, 32 Suppl 1, S235-S239 (2009-06-12)
The pyruvate dehydrogenase (PDH) complex is a mitochondrial multienzyme that catalyses the irreversible oxidative decarboxylation of pyruvate to acetyl-CoA. We report four novel PDHA1 mutations in patients with pyruvate dehydrogenase deficiency. Analysis of PDH activity showed decreased activity in fibroblasts

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