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Recombinant Human PDHA1 protein, His-tagged

Cat.No. : PDHA1-28668TH
Product Overview : Recombinant Human PDHA1 fussed with His tag was expressed in E. coli.
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Description : 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.
Source : E. coli
Species : Human
Tag : His
Form : Lyophilised. 0.5% Trehalose, 6M Urea, 100mM Sodium phosphate, 10mM Sodium chloride, pH 4.5.
AA Sequence : MRKMLAAVSRVLSGASQKPASRVLV ASRNFANDATFEIKKCDLHRLEEGP PVT TVLTREDGLKYYRMMQTVRRMELKA DQLYKQKIIRGFCHLCDGQE ACCVGLEA GINPTDHLITAYRAHGFTFTRGLSV REILAELTGRKGGCA KGKGGSMHMYAKN FYGGNGIVGAQVPLGAGIALACKYN GKDEVCLTLY GDGAANQGQIFEAYNMAA LWKLPCIFICENNRYGMGTSVERAA ASTDY YKRGDFIPGLRVDGMDILCVREA TRFAAAYCRSGKGPILMELQTYRYH GHSMSDPGVSYRTREEIQEVRSKSD PIM LLKDRMVNSNLASVEELKEI DVEVRKEIEDAAQFATADPEPPLEE LGYHIYSS DPPFEVRGANQWIKF KSVS
Applications : Western blot; Mass Spectrometry; SDS-PAGE
Stability : Upon delivery aliquot and store at -80 centigrade. Avoid freeze / thaw cycles.
Reconstitution : Reconstitute with 160 µl aqua dest.
Gene Name : PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 [ Homo sapiens ]
Official Symbol : PDHA1
Synonyms : PDHA; PDHAD; PHE1A; PDHCE1A; pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; pyruvate dehydrogenase E1 subunit; pyruvate dehydrogenase complex, E1-alpha polypeptide 1
Gene ID : 5160
mRNA Refseq : NM_000284
Protein Refseq : NP_000275
MIM : 300502
UniProt ID : P08559
Chromosome Location : Xp22.1
Pathway : Acetylcholine Synthesis, organism-specific biosystem; Biosynthesis of antibiotics, organism-specific biosystem; Central carbon metabolism in cancer, organism-specific biosystem
Function : pyruvate dehydrogenase (acetyl-transferring) activity; pyruvate dehydrogenase activity

For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.

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Q&As (5)

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What are the symptoms of PDHA1-related disorders? 01/28/2022

Symptoms may include developmental delay, intellectual disability, muscle weakness, and movement disorders.

What are the treatment options for PDHA1-related disorders? 12/01/2020

Treatment may involve dietary modifications, supplements, and supportive therapies to manage symptoms.

Are there any ongoing research efforts related to PDHA1 protein? 07/17/2019

Yes, researchers are studying potential gene therapy approaches and novel treatments for PDHA1-related disorders.

How does the PDHA1 protein impact brain function? 01/05/2018

The PDHA1 protein is critical for supplying energy to the brain, and mutations can lead to neurological impairments.

How does a mutation in the PDHA1 gene affect human health? 12/25/2017

Mutations in the PDHA1 gene can lead to pyruvate dehydrogenase complex deficiency, resulting in neurological and developmental problems.

Customer Reviews (3)

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Reviews
06/27/2020

    Incorporating the PDHA1 protein into your research will not only meet but exceed your expectations, enabling you to advance your scientific discoveries with confidence.

    02/28/2019

      Researchers can leverage the unique properties of the PDHA1 protein to gain insights into its biological functions and its impact on disease mechanisms.

      07/10/2017

        It has been shown to play a crucial role in multiple cellular processes, making it highly valuable for investigations into cell signaling, inflammation, and immune responses.

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