Mouse pyruvate dehydrogenase (lipoamide) beta ELISA Kit | PDHB elisa kit
Mouse Pyruvate dehydrogenase E1 component subunit beta, mitochondrial, PDHB ELISA Kit
NCBI and Uniprot Product Information
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: Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis; Carbohydrate Metabolism - pyruvate; Oxidoreductase; Carbohydrate Metabolism - glycolysis and gluconeogenesis; EC 1.2.4.1; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial; Carbohydrate Metabolism - butanoate
Cellular Component: nucleoplasm; mitochondrion; pyruvate dehydrogenase complex; nucleus
Molecular Function: pyruvate dehydrogenase activity; oxidoreductase activity; catalytic activity; pyruvate dehydrogenase (acetyl-transferring) activity
Biological Process: acetyl-CoA biosynthetic process from pyruvate; metabolic process; tricarboxylic acid cycle; carbohydrate metabolic process; glucose metabolic process
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Product Notes
The Mouse PDHB pdhb (Catalog #AAA9318208) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9318208 ELISA Kit recognizes Mouse PDHB. It is sometimes possible for the material contained within the vial of "pyruvate dehydrogenase (lipoamide) beta, ELISA Kit" 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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