{"product_id":"gbe1-polyclonal-antibody-bs8283","title":"GBE1 Polyclonal Antibody-BS8283","description":"\u003cp\u003eGBE1 Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50µl, 100µl\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: BS8283-50, BS8283-100\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: Q04446\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\t1:500 - 1:2000\u003c\/p\u003e\n\n\u003cp\u003eBackground: GBE1 (glucan (1,4-alpha-), branching enzyme 1) is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.\u003c\/p\u003e\n\n\u003cp\u003ePurification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is \u0026gt; 95% (by SDS-PAGE).\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: GBE1 polyclonal antibody detects endogenous levels of GBE1 protein.\u003c\/p\u003e\n\n\u003cp\u003eBioworld Molecular Weight: ~ 80 kDa\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 (WB) analysis of GBE1 polyclonal antibody\u003c\/p\u003e\n\n\u003cp\u003eAlternative Name: 1,4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme; GBE1\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Recombinant full length Human GBE1.\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":42923514986675,"sku":"BS8283-50","price":342.59,"currency_code":"CAD","in_stock":true},{"title":"100µl","offer_id":42923515019443,"sku":"BS8283-100","price":589.65,"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_96cefb34-8a0f-45f6-a331-eac8c569dff3.jpg?v=1703850900","url":"https:\/\/afsbio.com\/products\/gbe1-polyclonal-antibody-bs8283","provider":"AFSBio Inc.","version":"1.0","type":"link"}