CST, 9482S, Mitofusin-2 (D2D10) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying Mitofusin-2. Validated for Western Blotting,Immunohistochemistry (Paraffin),Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, Mitofusin-2 (D2D10) Rabbit Monoclonal Antibody (CST #9482) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunohistochemistry (Paraffin): 1:200 - 1:800 Immunofluorescence (Immunocytochemistry): 1:50 Storage Supplied 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. For a carrier free (BSA and azide free) version of this product see product # 92271 . Protocol Available protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Immunocytochemistry) Specificity / Sensitivity Mitofusin-2 (D2D10) Rabbit Monoclonal Antibody recognizes endogenous levels of total mitofusin-2 protein. Species Reactivity: Human, Mouse, Rat, Hamster, Monkey Source / Purification Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val573 of human mitofusin-2 protein. Background Mitofusins 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 neurogenerative disease characterized by a progressive loss of muscle tissue and sensory perception (5,6). Alternate Names CMT2A; CMT2A2; CMT2A2A; CMT2A2B; CPRP1; HMSN6A; HSG; hyperplasia suppressor; KIAA0214; MARF; MFN2; mitochondrial assembly regulatory factor; mitofusin 2; Mitofusin-2; Transmembrane GTPase MFN2 Specification REACTIVITY: H M R Hm Mk SENSITIVITY: Endogenous MW (kDa): 80 Source/Isotype: Rabbit IgG