Anti-Mitofusin 2/MFN2 Antibody Picoband® Fluoro647 Conjugated

Mitofusin-2 is a protein that in humans is encoded by the MFN2 gene. It is mapped to chromosome 1 and encodes a 757-amino acid protein that contains an ATP/GTP-binding site motif. This gene is expressed in many tissues and cell lines such as brain and KG-1 with the highest expression in heart and skeletal muscle. It has been found that MFN2 triggers mitochondrial energization, at least in part, by regulating OXPHOS expression through signals that are independent of its role as a mitochondrial fusion protein. And it contributes to the maintenance and operation of the mitochondrial network. Axonal CMT type 2A and autosomal dominant HMSN VI are caused by MFN2 and mutations in MFN2, which emphasizes its important role of mitochondrial function for both optic atrophies and peripheral neuropathies.

E.coli-derived human Mitofusin 2 recombinant protein (Position: V601-R757) . Human Mitofusin 2 shares 96% and 95% amino acid (aa) sequence identity with mouse and rat Mitofusin 2, respectively.

Apoptosis, Cancer, Cell Biology, Cell Death, Metabolism, Metabolism Processes, Mitochondria, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Neurodegenerative Disease, Neurology Process, Neuroscience, Organelles, Pathways and Processes, Signal Transduction, Subcellular Markers, Tags & Cell Markers

Essential transmembrane GTPase, which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. MFN2 acts independently of the cytoskeleton. It therefore plays a central role in mitochondrial metabolism and may be associated with obesity and/or apoptosis processes. Overexpression induces the formation of mitochondrial networks. Plays an important role in the regulation of vascular smooth muscle cell proliferation. Involved in the clearance of damaged mitochondria via selective autophagy (mitophagy) . Is required for PARK2 recruitment to dysfunctional mitochondria. Involved in the control of unfolded protein response (UPR) upon ER stress including activation of apoptosis and autophagy during ER stress. Acts as an upstream regulator of EIF2AK3 and suppresses EIF2AK3 activation under basal conditions. .

1. Neuspiel M, Zunino R, Gangaraju S, Rippstein P, McBride H. Activated mitofusin 2 signals mitochondrial fusion, interferes with Bax activation, and reduces susceptibility to radical induced depolarization. J Biol Chem. 2005 Jul 1; 280 (26) :25060-70. 2. Santel A, Fuller MT. Control of mitochondrial morphology by a human mitofusin. J Cell Sci. 2001 Mar; 114 (Pt 5) :867-74 3. Pich, S., Bach, D., Briones, P., Liesa, M., Camps, M., Testar, X., Palacin, M., Zorzano, A. The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system. Hum. Molec. Genet. 14: 1405-1415, 2005. 4. Zuchner S, De Jonghe P, Jordanova A, Claeys KG, Guergueltcheva V, Cherninkova S, Hamilton SR, Van Stavern G, Krajewski KM, Stajich J, Tournev I, Verhoeven K, Langerhorst CT, de Visser M, Baas F, Bird T, Timmerman V, Shy M, Vance JM. Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2. Ann Neurol. 2006 Feb; 59 (2) :276.