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PDHA1 Polyclonal Antibody, 100μg, (ATB-T3641)
Background: 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]Product datasheet: Overview Product Description PDHA1 Polyclonal Antibody, 100μg, (ATB-T3641) Image Species Reactivities Human,Mouse,Rat Immunogen Synthesized peptide derived from the C-terminal region of human PDHA1. Properties Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Instructions-20°C/1 year References: Tyr-301 phosphorylation inhibits pyruvate dehydrogenase by blocking substrate binding and promotes the Warburg effect. Fan J, et al. J Biol Chem, 2014 Sep 19. PMID 25104357 Intermittent-relapsing pyruvate dehydrogenase complex deficiency: a case with clinical, biochemical, and neuroradiological reversibility. Giribaldi G, et al. Dev Med Child Neurol, 2012 May. PMID 22142326 Molecular characterization of 82 patients with pyruvate dehydrogenase complex deficiency. Structural implications of novel amino acid substitutions in E1 protein. Imbard A, et al. Mol Genet Metab, 2011 Dec. PMID 21914562 Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency. Sharma R, et al. Dev Med Child Neurol, 2012 May. PMID 21895644 MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency. Ah Mew N, et al. Pediatr Neurol, 2011 Jul. PMID 21723463 Free PMC Article
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IntelixBio has swiftly become a household name in the medical industry, primarily owing to its cutting-edge medical lab equipment. Our commitment to excellence resonates through every facet of our products, ensuring that laboratories worldwide receive top-tier solutions for their diagnostic needs. Our medical lab equipment is engineered with precision and innovation, embodying the pinnacle of technological advancement in the field. From state-of-the-art analyzers to automated specimen-handling systems, our extensive catalog caters to diverse laboratory requirements, fostering efficiency and accuracy in every diagnostic process. Laboratories entrust IntelixBio's medical lab equipment for its unwavering reliability, empowering healthcare professionals with the tools they need to deliver optimal patient care.
At IntelixBio, our dedication to advancing healthcare is exemplified through our unparalleled range of medical lab equipment. Each product is meticulously crafted to meet the exacting standards of modern laboratory environments, facilitating seamless integration and operation. Our commitment to quality permeates every aspect of our offerings, ensuring that laboratories can consistently rely on our medical lab equipment to deliver precise and dependable results. With a comprehensive selection that spans from basic laboratory essentials to sophisticated diagnostic instruments, IntelixBio remains the foremost choice for institutions seeking excellence in medical equipment solutions. Trust IntelixBio to elevate your laboratory capabilities with our innovative medical lab equipment, empowering you to drive progress in healthcare delivery.
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At IntelixBio LLC, we offer high-quality medical lab equipment. Our lab supplies include:
- Protective gear
- Antibody immunodetection supplies
- Bioreagents
- Lab Supplies
- Immunohistochemistry Tools
- Instruments and Equipment
- PPE
- Sample preparation supplies
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