Rabbit PDHB Polyclonal Antibody | anti-PDHB antibody
PDHB Rabbit Polyclonal
Immunogen Affinity Purified
Immunogen Affinity Purified
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 carbon dioxide, 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 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]
Uniprot Description
PDHB: the beta subunit of pyruvate dehydrogenase (PDH), 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. Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency), the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS). Two alternatively spliced human isoforms have been described.
Protein type: Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - butanoate; EC 1.2.4.1; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 3p21.1-p14.2
Cellular Component: mitochondrial matrix; mitochondrion; nucleoplasm; nucleus; pyruvate dehydrogenase complex
Molecular Function: protein binding; pyruvate dehydrogenase (acetyl-transferring) activity; pyruvate dehydrogenase activity
Biological Process: acetyl-CoA biosynthetic process from pyruvate; glucose metabolic process; glyoxylate metabolic process; pyruvate metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; tricarboxylic acid cycle
Disease: Pyruvate Dehydrogenase E1-beta Deficiency
Research Articles on PDHB
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Product Notes
The PDHB pdhb (Catalog #AAA7602503) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PDHB Rabbit Polyclonal reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's PDHB can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunoprecipitation (IP). Researchers should empirically determine the suitability of the PDHB pdhb 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 "PDHB, 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
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