{"product_id":"new-product-225441","title":"GBE1 polyclonal antibody","description":"\u003cp\u003eGBE1 (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","brand":"Bioworld","offers":[{"title":"100ul \/ Rabbit \/ Human,Mouse,Rat","offer_id":47661850755352,"sku":"BS8283","price":408.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/BIOWORLD_c319a43b-cde9-44d9-9832-bb18cdd23dad.jpg?v=1707469279","url":"https:\/\/danabiosci.com\/products\/new-product-225441","provider":"Dana Bioscience","version":"1.0","type":"link"}