WB, IHC-P, IF-IC
H M R Hm Mk
Endogenous
80
Rabbit IgG
#O95140
9927
Product Information
Product Usage Information
This formulation is ideal for use with technologies requiring specialized or custom antibody labeling, including fluorophores, metals, lanthanides, and oligonucleotides. It is not recommended for ChIP, ChIP-seq, CUT&RUN or CUT&Tag assays. If you require a carrier free formulation for chromatin profiling, please contact us. Optimal dilutions/concentrations should be determined by the end user.
Formulation
Storage
Specificity / Sensitivity
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).
- 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.
Species Reactivity
Species reactivity is determined by testing in at least one approved application (e.g., western blot).
Applications Key
WB: Western Blotting IHC-P: Immunohistochemistry (Paraffin) IF-IC: Immunofluorescence (Immunocytochemistry)
Cross-Reactivity Key
H: human M: mouse R: rat Hm: hamster Mk: monkey Vir: virus Mi: mink C: chicken Dm: D. melanogaster X: Xenopus Z: zebrafish B: bovine Dg: dog Pg: pig Sc: S. cerevisiae Ce: C. elegans Hr: horse GP: Guinea Pig Rab: rabbit All: all species expected
Trademarks and Patents
限制使用
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