Mitofusin-2 (D1E9) Rabbit mAb #11925
- WB
- IP
- IF
Supporting Data
| REACTIVITY | H Hm Mk |
| SENSITIVITY | Endogenous |
| MW (kDa) | 80 |
| Source/Isotype | Rabbit IgG |
Application Key:
- WB-Western Blotting
- IP-Immunoprecipitation
- IF-Immunofluorescence
Species Cross-Reactivity Key:
- H-Human
- Hm-Hamster
- Mk-Monkey
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
| Immunoprecipitation | 1:200 |
| Immunofluorescence (Immunocytochemistry) | 1:100 - 1:200 |
Storage
Protocol
Specificity / Sensitivity
Mitofusin-2 (D1E9) Rabbit mAb recognizes endogenous levels of total mitofusin-2 protein.
Species Reactivity:
Human, 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 neurodegenerative 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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