Recombinant Human PDHA1 Protein, Myc/DDK-tagged, C13 and N15-labeled
Cat.No. : | PDHA1-1310H |
Product Overview : | PDHA1 MS Standard C13 and N15-labeled recombinant protein (NP_000275) with a C-terminal MYC/DDK tag, was expressed in HEK293 cells. |
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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 : | HEK293 |
Species : | Human |
Tag : | Myc/DDK |
Molecular Mass : | 43.3 kDa |
AA Sequence : | MRKMLAAVSRVLSGASQKPASRVLV ASRNFANDATFEIKKCDLHRLEEGP PVTTVLTREDGLKYYRMMQTVRRME LKADQLYKQKIIRGFCHLCDGQEAC CVGLEAGINPTDHLITAYRAHGFTF TRGLSVREILAELTGRKGGCAKGKG GSMHMYAKNFYGGNGIVGAQVPLGA GIALACKYNGKDEVCLTLYGDGAAN QGQIFEAYNMAALWKLPCIFICENN RYGMGTSVERAAASTDYYKRGDFIP GLRVDGMDILCVREATRFAAAYCRS GKGPILMELQTYRYHGHSMSDPGVS YRTREEIQEVRSKSDPIMLLKDRMV NSNLASVEELKEIDVEVRKEIEDAA QFATADPEPPLEELGYHIYSSDPPF EVRGANQWIKFKSVSTRTRPLEQKL ISEEDLAANDILDYKDDDDKV |
Purity : | > 80% as determined by SDS-PAGE and Coomassie blue staining |
Stability : | Stable for 3 months from receipt of products under proper storage and handling conditions. |
Storage : | Store at -80 centigrade. Avoid repeated freeze-thaw cycles. |
Concentration : | 50 μg/mL as determined by BCA |
Storage Buffer : | 100 mM glycine, 25 mM Tris-HCl, pH 7.3. |
Gene Name : | PDHA1 pyruvate dehydrogenase E1 subunit alpha 1 [ Homo sapiens (human) ] |
Official Symbol : | PDHA1 |
Synonyms : | PDHA1; pyruvate dehydrogenase (lipoamide) alpha 1; PDHA; pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; pyruvate dehydrogenase complex, E1-alpha polypeptide 1; PHE1A; PDHCE1A; |
Gene ID : | 5160 |
mRNA Refseq : | NM_000284 |
Protein Refseq : | NP_000275 |
MIM : | 300502 |
UniProt ID : | P08559 |
Products Types
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PDHA1-825H | Recombinant Human PDHA1 Protein, MYC/DDK-tagged | +Inquiry |
Pdha1-4760M | Recombinant Mouse Pdha1 Protein, Myc/DDK-tagged | +Inquiry |
◆ Lysates | ||
PDHA1-001HCL | Recombinant Human PDHA1 cell lysate | +Inquiry |
Related Gene
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)
Ask a questionSymptoms may include developmental delay, intellectual disability, muscle weakness, and movement disorders.
Treatment may involve dietary modifications, supplements, and supportive therapies to manage symptoms.
Yes, researchers are studying potential gene therapy approaches and novel treatments for PDHA1-related disorders.
The PDHA1 protein is critical for supplying energy to the brain, and mutations can lead to neurological impairments.
Mutations in the PDHA1 gene can lead to pyruvate dehydrogenase complex deficiency, resulting in neurological and developmental problems.
Customer Reviews (3)
Write a reviewIncorporating the PDHA1 protein into your research will not only meet but exceed your expectations, enabling you to advance your scientific discoveries with confidence.
Researchers can leverage the unique properties of the PDHA1 protein to gain insights into its biological functions and its impact on disease mechanisms.
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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