{"product_id":"hmbs-polyclonal-antibody-bs90649","title":"HMBS Polyclonal Antibody-BS90649","description":"\u003cp\u003eHMBS Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50µl, 100µl\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: BS90649-50, BS90649-100\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: P08397(Human) P22907(Mouse) P19356(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\u003c\/p\u003e\n\n\u003cp\u003eAll Applications: WB:1:1,000-1:2,000\u003c\/p\u003e\n\n\u003cp\u003eBackground: PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.\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\u003eNote: For research use only, not for use in diagnostic procedure.\u003c\/p\u003e\n\n\u003cp\u003eExtra Notes: Western blot analysis of HMBS on differentLysates using anti-HMBS antibody at 1\/1,000 dilution. Positive control: Lane 1: Hela Lane 2: 293T\u003c\/p\u003e\n\n\u003cp\u003eBioworld Molecular Weight: 40 kDa\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: HMBS polyclonal antibody detects endogenous levels of HMBS protein.\u003c\/p\u003e\n\n\u003cp\u003eAlternative Name: PBG-D, HMBS, HEM3, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, PORC, Porphobilinogen deaminase, porphyria, acute; Chester type, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase,\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","brand":"BioWorld","offers":[{"title":"50µl","offer_id":42939200340147,"sku":"BS90649-50","price":415.38,"currency_code":"CAD","in_stock":true},{"title":"100µl","offer_id":42939200372915,"sku":"BS90649-100","price":640.78,"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_5f91ff83-4836-4927-aca9-eb586da8a6f0.jpg?v=1704545015","url":"https:\/\/afsbio.com\/products\/hmbs-polyclonal-antibody-bs90649","provider":"AFSBio Inc.","version":"1.0","type":"link"}