Western Blot (WB)
(WB Suggested Anti-PDHA1 AntibodyPositive Control: Lane 1: 60ug human NT2 cell linePrimary Antibody Dilution : 1:500Secondary Antibody : IRDye 800 CW goat anti-rabbit from Li-COR BioscienceSecondry Antibody Dilution : 1:20,000Submitted by: Dr. Yuzhi Chen, University of Arkansas for Medical Science)
Rabbit PDHA1 Polyclonal Antibody | anti-PDHA1 antibody
PDHA1 antibody - C-terminal region
Western Blot (WB)
(WB Suggested Anti-PDHA1 AntibodyPositive Control: Lane 1: 60ug human NT2 cell linePrimary Antibody Dilution : 1:500Secondary Antibody : IRDye 800 CW goat anti-rabbit from Li-COR BioscienceSecondry Antibody Dilution : 1:20,000Submitted by: Dr. Yuzhi Chen, University of Arkansas for Medical Science)
Western Blot (WB)
(WB Suggested Anti-PDHA1 AntibodyPositive Control: Lane 1: 60ug human NT2 cell linePrimary Antibody Dilution : 1:500Secondary Antibody : IRDye 800 CW goat anti-rabbit from Li-COR BioscienceSecondry Antibody Dilution : 1:20,000Submitted by: Dr. Yuzhi Chen, University of Arkansas for Medical Science)
Target Description: 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.
NCBI and Uniprot Product Information
NCBI 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.[provided by RefSeq, Mar 2010]
Uniprot Description
PDHA1: a mitochondrial matrix enzyme that catalyzes the oxidative decarboxylation of pyruvate, producing acetyl-CoA and CO2. A key enzyme in controlling the balance between lipid and glucose oxidation depending on substrate availability. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. The activity of PDH is tightly regulated by phosphorylation. The phosphorylation of at least one of three specific serine residues in E1 subunit by PDHK inactivates the PDHC, while dephosphorylation by PDP restores its activity. Sites 1, 2, and 3 of PDHA1 are S293, S300, and S232, respectively. Four PDHK isoenzymes have been described, each with different site specificity: all four phosphorylate sites 1 and 2 but at different rates; for site 1 PDHK2 >PDHK4 >PDHK1 >PDHK3; for site 2, PDHK3> PDHK4 > PDHK2 > PDHK1. Only PDHK1 phosphorylates site 3. PDHA1 deficiency is the most common enzyme defect in patients with primary lactic acidosis.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - butanoate; Oxidoreductase; EC 1.2.4.1; Carbohydrate Metabolism - pyruvate; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis
Chromosomal Location of Human Ortholog: Xp22.1
Cellular Component: mitochondrion; mitochondrial matrix; pyruvate dehydrogenase complex; nucleus
Molecular Function: pyruvate dehydrogenase activity; pyruvate dehydrogenase (acetyl-transferring) activity
Biological Process: cellular metabolic process; acetyl-CoA biosynthetic process from pyruvate; glycolysis; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process
Disease: Pyruvate Dehydrogenase E1-alpha Deficiency
Research Articles on PDHA1
Similar Products
Product Notes
The PDHA1 pdha1 (Catalog #AAA3208870) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PDHA1 antibody - C-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Pig, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's PDHA1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the PDHA1 pdha1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PDHA1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
