Product Pathways - Metabolism
Mitofusin-2 (D2D10) Rabbit mAb #9482
|W IHC-P IF-IC||H M R Hm Mk||Endogenous||80||Rabbit IgG|
Reactivity Key: H=Human M=Mouse R=Rat Hm=Hamster Mk=Monkey
Species cross-reactivity is determined by western blot. Species enclosed in parentheses are predicted to react based on 100% sequence homology.
Specificity / Sensitivity
Mitofusin-2 (D2D10) Rabbit mAb recognizes endogenous levels of total mitofusin-2 protein.
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val573 of human mitofusin-2 protein.
Western blot analysis of extracts from various cell lines using Mitofusin-2 (D2D10) Rabbit mAb.
Immunohistochemical analysis of paraffin-embedded human colon carcinoma using Mitofusin-2 (D2D10) Rabbit mAb.
Immunohistochemical analysis of paraffin-embedded mouse heart using Mitofusin-2 (D2D10) Rabbit mAb.
Immunohistochemical analysis of paraffin-embedded human ovarian carcinoma using Mitofusin-2 (D2D10) Rabbit mAb in the presence of control peptide (left) or antigen-specific peptide (right).
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).
- Zhang, Y. and Chan, D.C. (2007) FEBS Lett 581, 2168-73.
- Chan, D.C. (2006) Annu Rev Cell Dev Biol 22, 79-99.
- Chen, H. et al. (2010) Cell 141, 280-9.
- Bereiter-Hahn, J. and Vöth, M. (1994) Microsc Res Tech 27, 198-219.
- Kijima, K. et al. (2005) Hum Genet 116, 23-7.
- Züchner, S. et al. (2004) Nat Genet 36, 449-51.
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For Research Use Only. Not For Use In Diagnostic Procedures.