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PDHA1 / PDH E1 Alpha

pyruvate dehydrogenase (lipoamide) alpha 1

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Gene Name: pyruvate dehydrogenase (lipoamide) alpha 1
Synonyms: PDHA1, PDHCE1A, PDHA, PHE1A, PDHE1-A type I
Target Sequences: NM_000284 NP_000275.1 P08559

Publications (8)

1
Pyruvate dehydrogenase complex activity controls metabolic and malignant phenotype in cancer cells. McFate T, Mohyeldin A, Lu H, Thakar J, Henriques J, Halim ND, Wu H, Schell MJ, Tsang TM, Teahan O, Zhou S, Califano JA, Jeoung NH, Harris RA, Verma A. The Journal of biological chemistry. 2008 283:22700-8. [PubMed:18541534] [PMC:PMC2504897]
2
Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasis. Acin-Perez R, Hoyos B, Zhao F, Vinogradov V, Fischman DA, Harris RA, Leitges M, Wongsiriroj N, Blaner WS, Manfredi G, Hammerling U. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2010 24:627-36. [PubMed:19812372] [PMC:PMC2812036]
3
Regulation of intermediary metabolism by the PKCdelta signalosome in mitochondria. Acin-Perez R, Hoyos B, Gong J, Vinogradov V, Fischman DA, Leitges M, Borhan B, Starkov A, Manfredi G, Hammerling U. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2010 24:5033-42. (WB; Mouse) [PubMed:20798245] [PMC:PMC2992363]
4
Erk regulation of pyruvate dehydrogenase flux through PDK4 modulates cell proliferation. Grassian AR, Metallo CM, Coloff JL, Stephanopoulos G, Brugge JS. Genes & development. 2011 25:1716-33. [PubMed:21852536] [PMC:PMC3165936]
5
p53 negatively regulates transcription of the pyruvate dehydrogenase kinase Pdk2. Contractor T, Harris CR. Cancer research. 2012 72:560-7. [PubMed:22123926]
6
Two protein kinase C isoforms, and , regulate energy homeostasis in mitochondria by transmitting opposing signals to the pyruvate dehydrogenase complex. Gong J, Hoyos B, Acin-Perez R, Vinogradov V, Shabrova E, Zhao F, Leitges M, Fischman D, Manfredi G, Hammerling U. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2012 26:3537-49. (WB; Mouse) [PubMed:22573912] [PMC:PMC3405274]
7
The novel function of tumor protein D54 in regulating pyruvate dehydrogenase and metformin cytotoxicity in breast cancer. Zhuang Y, Ly RC, Frazier CV, Yu J, Qin S, Fan XY, Goetz MP, Boughey JC, Weinshilboum R, Wang L. Cancer & metabolism. 2019 January;7:1. [Full Text Article] [PubMed:30697423] [PMC:PMC6345044]
8
Pyruvate dehydrogenase alpha 1 as a target of omega-3 polyunsaturated fatty acids in human prostate cancer through a global phosphoproteomic analysis. Heng Zhao, Beth R Pflug, Xianyin Lai, Mu Wang. Proteomics. 2016 September;16:2419-31. [Full Text Article] [PubMed:27357730]
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Proteins (11)
Over-Expression Lysate (8)
Recombinant (3)
PDHA1 / PDH E1 Alpha (11)
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GST (1)
His (1)
Myc-DDK (Flag) (9)
Human (7)
293T Cells (4)
E. coli (1)
HEK 293 Cells (5)
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Purified (1)
PDHA1 / PDH E1 Alpha Protein - Recombinant Pyruvate Dehydrogenase Alpha By SDS-PAGE
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E. coli
His
41.7 kDa
10 µg/$281; 50 µg/$442; 100 µg/$707; 200 µg/$885; 1 mg/$1,994; 500 µg/$1,534; 5 mg/$3,095; 2 mg/$2,181
PDHA1 / PDH E1 Alpha Protein - 12.5% SDS-PAGE of human PDHA1 stained with Coomassie Blue
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Wheat Germ Extract
GST
68.64 kDa
10 µg/$479; 25 µg/$670
PDHA1 / PDH E1 Alpha Protein - Purified recombinant protein PDHA1 was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
40.2 kDa
20 µg/$1,107
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
44.6 kDa
100 µg/$494
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
40.6 kDa
100 µg/$494
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
48 kDa
100 µg/$494
Select
293T Cells
Myc-DDK (Flag)
43.3 kDa
20 µg/$150
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
48 kDa
20 µg/$150
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
44.6 kDa
20 µg/$150
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
40.6 kDa
20 µg/$150
PDHA1 / PDH E1 Alpha Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
43.3 kDa
100 µg/$494
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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).