{"product_id":"bbs2-rabbit-polyclonal-antibody-es18093","title":"BBS2 Rabbit Polyclonal Antibody - ES18093","description":"\u003cp\u003eBBS2 Rabbit Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES18093-50, ES18093-100\u003c\/p\u003e\n\n\u003cp\u003eLead times: 1-2 weeks, if manufacturer has product in stock\u003c\/p\u003e\n\n\u003cp\u003eManufacturer\/Ship Location: China\u003c\/p\u003e\n\n\u003cp\u003eBackground: This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, obesity, polydactyly, renal malformation and mental retardation. The proteins encoded by BBS gene family members are structurally diverse and the similar phenotypes exhibited by mutations in BBS gene family members is likely due to their shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene forms a multiprotein BBSome complex with seven other BBS proteins.[provided by RefSeq, Oct 2014],\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthesized peptide derived from human BBS2 AA range: 149-199\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eTarget: BBS2\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: This antibody detects endogenous levels of BBS2 at Human\/Mouse\/Rat\u003c\/p\u003e\n\n\u003cp\u003eDilution: WB 1：500-2000\u003c\/p\u003e\n\n\u003cp\u003ePurification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml,\u003c\/p\u003e\n\n\u003cp\u003eFormlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.\u003c\/p\u003e\n\n\u003cp\u003eExpression: Widely expressed,\u003c\/p\u003e\n\n\u003cp\u003eMolecular Weight (Da): 79kD\u003c\/p\u003e\n\n\u003cp\u003eFunction: Disease: Defects in BBS2 are the cause of Bardet-Biedl syndrome type 2 (BBS2) [MIM: 209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect\u003cbr\u003e\nFunction: The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP\/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Cell projection, cilium membrane Cytoplasm Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eGene Name: BBS2\u003c\/p\u003e\n\n\u003cp\u003eProtein Name: BBS2\u003c\/p\u003e\n\n\u003cp\u003eMouse Gene ID: 67378\u003c\/p\u003e\n\n\u003cp\u003eMouse SwissProt Number: Q9CWF6\u003c\/p\u003e\n\n\u003cp\u003eRat Gene ID: 113948\u003c\/p\u003e\n\n\u003cp\u003eRat SwissProt Number: Q99MH9\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":46315758256307,"sku":"ES18093-50","price":186.0,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":46315758289075,"sku":"ES18093-100","price":372.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/elk_antibodies.jpg?v=1793190497","url":"https:\/\/afsbio.com\/products\/bbs2-rabbit-polyclonal-antibody-es18093","provider":"AFSBio Inc.","version":"1.0","type":"link"}