PDHA1
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Official Full Name
pyruvate dehydrogenase (lipoamide) alpha 1
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Overview
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. -
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;
- Recombinant Proteins
- Cell & Tissue Lysates
- Protein Pre-coupled Magnetic Beads
- Chicken
- Human
- Mouse
- Rhesus Macaque
- E.coli
- HEK293
- HEK293T
- Insect Cell
- Mammalian Cell
- Mammalian cells
- Flag
- GST
- His
- His (Fc)
- Avi
- Myc
- DDK
- Myc|DDK
- N/A
- N
- Involved Pathway
- Protein Function
- Interacting Protein
- PDHA1 Related Research Area
PDHA1 involved in several pathways and played different roles in them. We selected most pathways PDHA1 participated on our site, such as Glycolysis / Gluconeogenesis, Citrate cycle (TCA cycle), Pyruvate metabolism, which may be useful for your reference. Also, other proteins which involved in the same pathway with PDHA1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
Pathway Name | Pathway Related Protein |
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Glycolysis / Gluconeogenesis | ADH1C;MINPP1A;HK3;ENO1B;ADH2-2;ALDH1A3;ADH1B;GPI;PGAM1B |
Citrate cycle (TCA cycle) | OGDH;DLST;DLD;IDH2;MDH1AA;OGDHA;SUCLG1;SDHA;FH |
Pyruvate metabolism | ALDH3A2A;PDHA1B;ME2;BRP44;FH;MDH1;SLC16A1;MPC1;ACYP2 |
Metabolic pathways | POLE2;AGK;ARSB;CYP3A7;ADH6;DUT;POLR2G;PTDSS2;ATP6V0E |
Carbon metabolism | ACADS;PSAT1;ADPGK;PGAM1A;PRPS1L1;TPI1B;MTHFR;PKMB;PRPS1 |
HIF- signaling pathway | HK2;STAT3;RPS6KB2;ARNT;TCEB1;CAMK2A;INS;FLT1;IFNGR2 |
Central carbon metabolism in cancer | LDHA;SLC2A1;PDGFRA;PIK3CD;MAP2K2;FGFR2;PDGFRB;SLC16A3;PIK3CG |
PDHA1 has several biochemical functions, for example, pyruvate dehydrogenase (acetyl-transferring) activity, pyruvate dehydrogenase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by PDHA1 itself. We selected most functions PDHA1 had, and list some proteins which have the same functions with PDHA1. You can find most of the proteins on our site.
Function | Related Protein |
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pyruvate dehydrogenase (acetyl-transferring) activity | PDHA1B;PDHA1A;PDHA1;PDHA2;PDHB |
pyruvate dehydrogenase activity | PDHA1;PDHB |
PDHA1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with PDHA1 here. Most of them are supplied by our site. Hope this information will be useful for your research of PDHA1.
PDHB; PDK1; PDP1; USP19; DGKE; WDR20; BABAM1; PRKAB1; ITGB3BP; 15-deoxy-delta(12,14; bosutinib; VCAM1; STAT5A; STAT5B; TEAD3; HDAC6; Hdac6
- Q&As
- Reviews
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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