{"product_id":"cst-11925s","title":"CST,  11925S, Mitofusin-2 (D1E9) Rabbit Monoclonal Antibody","description":"Monoclonal Antibody for studying Mitofusin-2. Validated for Western Blotting,Immunoprecipitation,Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, Mitofusin-2 (D1E9) Rabbit Monoclonal Antibody (CST #11925) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\nImmunoprecipitation: 1:200\nImmunofluorescence (Immunocytochemistry): 1:100 - 1:200\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting, Immunoprecipitation, Immunofluorescence (Immunocytochemistry)\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nMitofusin-2 (D1E9) Rabbit Monoclonal Antibody recognizes endogenous levels of total mitofusin-2 protein.\nSpecies Reactivity: Human, Hamster, Monkey\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val573 of human mitofusin-2 protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nMitofusins are mitochondrial transmembrane GTPases that function to regulate mitochondrial fusion, a process that occurs in concert with mitochondrial division and is necessary for the maintenance of structural and genetic mitochondrial integrity (1,2). Two mitofusins have been described in mammals, mitofusin-1 and -2, which share 60% amino acid identity and appear to function coordinately to regulate mitochondrial fusion (3). Mitochondrial fusion is widely recognized as important for normal cell growth and development (4), and may have evolved as a mechanism to offset the deleterious effects of mtDNA mutations (3). Null mutations in either mitofusin are embryonic lethal in mice, whereas conditional knockout studies have shown that combined deletion of mitofusin-1 and mitofusin-2 in skeletal muscle results in severe mitochondrial dysfunction (3). Research studies have revealed that mutations in mitofusin-2 are linked to Charcot-Marie-Tooth disease, an inherited neurodegenerative disease characterized by a progressive loss of muscle tissue and sensory perception (5,6).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nCMT2A; CMT2A2; CMT2A2A; CMT2A2B; CPRP1; HMSN6A; HSG; hyperplasia suppressor; KIAA0214; MARF; MFN2; mitochondrial assembly regulatory factor; mitofusin 2; Mitofusin-2; Transmembrane GTPase MFN2\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H Hm Mk\nSENSITIVITY: Endogenous\nMW (kDa): 80\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46797561659561,"sku":"11925S","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-11925s","provider":"Iright","version":"1.0","type":"link"}