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Merck
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Key Documents

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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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

rabbit

品質等級

共軛

unconjugated

抗體表格

IgG fraction of antiserum

抗體產品種類

primary antibodies

無性繁殖

polyclonal

形狀

buffered aqueous solution

分子量

40 kDa

物種活性

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

濃度

0.5 mg - 1 mg/mL

技術

western blot: suitable

NCBI登錄號

UniProt登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... PDHA1(5160)

一般說明

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

應用

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.

序列

Synthetic peptide located within the following region: MRKMLAAVSRVLSGASQKPASRVLVASRNFANDATFEIKKCDLHRLEEGP

外觀

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

免責聲明

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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儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

WGK 3

閃點(°F)

Not applicable

閃點(°C)

Not applicable


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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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