{"product_id":"pdhb-polyclonal-antibody-bs91041","title":"PDHB Polyclonal Antibody-BS91041","description":"\u003cp\u003ePDHB Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50µl, 100µl\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: BS91041-50, BS91041-100\u003c\/p\u003e\n\n\u003cp\u003eProduct: Rabbit IgG, 1mg\/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2\u003c\/p\u003e\n\n\u003cp\u003eSwiss-Prot: P11177(Human) Q9D051(Mouse) P49432(Rat)\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB, IHC, IP, FC\u003c\/p\u003e\n\n\u003cp\u003eAll Applications: WB:1:500-1:2,000\u003cbr\u003e\nIHC:1:50-1:200\u003cbr\u003e\nFC:1:50-1:100\u003c\/p\u003e\n\n\u003cp\u003eBackground: The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency). PDHE1 deficiency is 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) (Leigh encephalomyelopathy).\u003c\/p\u003e\n\n\u003cp\u003ePurification and Purity: ProA affinity purified\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze \/ thaw cycles.\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: PDHB polyclonal antibody detects endogenous levels of PDHB protein.\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Recombinant protein\u003c\/p\u003e\n\n\u003cp\u003eConjugate: Unconjugated\u003c\/p\u003e\n\n\u003cp\u003eModification: Unmodified\u003c\/p\u003e\n\n\u003cp\u003eNote: For research use only, not for use in diagnostic procedure.\u003c\/p\u003e\n\n\u003cp\u003eExtra Notes: Western blot analysis of Pyruvate Dehydrogenase E1 beta subunit on HepG2 (1) and Hela (2) cell lysate using anti-Pyruvate Dehydrogenase E1 beta subunit antibody at 1\/1,000 dilution., Western blot analysis of Pyruvate Dehydrogenase E1 beta subunit on mouse kidney tissue (1) and PC-12 (2) cell lysate using anti-Pyruvate Dehydrogenase E1 beta subunit antibody at 1\/1,000 dilution.\u003c\/p\u003e","brand":"BioWorld","offers":[{"title":"50µl","offer_id":42892365365427,"sku":"BS91041-50","price":424.94,"currency_code":"CAD","in_stock":true},{"title":"100µl","offer_id":42892365398195,"sku":"BS91041-100","price":655.54,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/products\/thumbnail_d1447bac803ec9be_11-14-09-19-51_c6b921e4-f1a3-4510-a849-10d877e982cc.jpg?v=1702483911","url":"https:\/\/afsbio.com\/products\/pdhb-polyclonal-antibody-bs91041","provider":"AFSBio Inc.","version":"1.0","type":"link"}