Revision 1
#35121
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For Research Use Only. Not for Use in Diagnostic Procedures.
Applications:
W, IP
Reactivity:
H M R Mk
Sensitivity:
Endogenous
MW (kDa):
150
Source/Isotype:
Rabbit IgG
UniProt ID:
#Q99700
Entrez-Gene Id:
6311
Product Usage Information
Application | Dilution |
---|---|
Western Blotting | 1:1000 |
Immunoprecipitation | 1:50 |
Storage
Specificity/Sensitivity
Ataxin-2 (E3B3Z) Rabbit mAb recognizes endogenous levels of total ataxin-2 protein. This antibody may recognize a non-specific band of unknown origin at 18 kDa in rodent samples.
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val1055 of human ataxin-2 protein.
Background
Spinocerebellar ataxia type 2 (SCA2), a lethal autosomal dominant neurodegenerative disorder, is characterized by slurred speech, loss of limb coordination, and gait abnormalities resulting from the degeneration of cerebellar Purkinje cells and a subset of brainstem neurons (1,2). SCA2 is caused by an excessive expansion of polyglutamine (polyQ) repeats at the N-terminal coding region of the ATXN2 gene, which encodes the protein ataxin-2 (2). Intermediate-length polyQ repeats in ATXN2 have also been identified as a risk factor for amyotrophic lateral sclerosis (ALS) (3-5). Ataxin-2 is a ubiquitously expressed RNA-binding protein (RBP) that plays an important role in RNA stability and translation (6,7). Ataxin-2 can undergo liquid-liquid phase separation and is frequently recruited to cytoplasmic foci known as stress granules (SGs), which are ribonucleoprotein (RNP) granules formed at sites of stalled mRNA translation (8,9). Ataxin-2 has also been shown to promote the assembly of neuronal RNP granules necessary for long-term memory formation (10). It is hypothesized that the expanded polyQ repeats in mutant ataxin-2 promote aberrant protein aggregation and degeneration in Purkinje neurons. Indeed, ataxin-2 has been shown to interact with TDP43, another RBP that is frequently associated with pathological aggregates and inclusion bodies in ALS and frontotemporal dementia (FTD) (3,11-14). It is currently unclear if mutant ataxin-2 drives neurodegeneration through toxic gain-of-function or loss of physiological function, and more research is needed in this area (15). However, targeting ataxin-2 therapeutically has shown initial promise, as antisense oligonucleotides against ataxin-2 improve motor function in SCA2 mouse models and increase survival in ALS mouse models (16,17).
Background References
- Magaña, J.J. et al. (2013) Mol Neurobiol 47, 90-104.
- Laffita-Mesa, J.M. et al. (2021) Curr Opin Neurol 34, 578-588.
- Elden, A.C. et al. (2010) Nature 466, 1069-75.
- Sproviero, W. et al. (2017) Neurobiol Aging 51, 178.e1-178.e9.
- Glass, J.D. et al. (2022) Brain 145, 2671-2676.
- Yokoshi, M. et al. (2014) Mol Cell 55, 186-98.
- Inagaki, H. et al. (2020) J Biol Chem 295, 15810-15825.
- Ralser, M. et al. (2005) J Mol Biol 346, 203-14.
- Nonhoff, U. et al. (2007) Mol Biol Cell 18, 1385-96.
- Bakthavachalu, B. et al. (2018) Neuron 98, 754-766.e4.
- Neumann, M. et al. (2006) Science 314, 130-3.
- Liu-Yesucevitz, L. et al. (2010) PLoS One 5, e13250.
- Hart, M.P. and Gitler, A.D. (2012) J Neurosci 32, 9133-42.
- Watanabe, R. et al. (2020) Acta Neuropathol Commun 8, 176.
- Ostrowski, L.A. et al. (2017) Genes (Basel) 8, 157. doi: 10.3390/genes8060157.
- Scoles, D.R. et al. (2017) Nature 544, 362-366.
- Becker, L.A. et al. (2017) Nature 544, 367-371.
Species Reactivity
Species reactivity is determined by testing in at least one approved application (e.g., western blot).
Western Blot Buffer
IMPORTANT: For western blots, incubate membrane with diluted primary antibody in 5% w/v BSA, 1X TBS, 0.1% Tween® 20 at 4°C with gentle shaking, overnight.
Applications Key
W: Western Blotting IP: Immunoprecipitation
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 G: Goat All: All Species Expected
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Revision 1
#35121
Ataxin-2 (E3B3Z) Rabbit mAb
Western blot analysis of extracts from various cell lines using Ataxin-2 (E3B3Z) Rabbit mAb.
Western blot analysis of extracts from various cell lines and tissue using Ataxin-2 (E3B3Z) Rabbit mAb.
Immunoprecipitation of ataxin-2 protein from HeLa cell extracts. Lane 1 is 10% input, lane 2 is Rabbit (DA1E) mAb IgG XP® Isotype Control #3900, and lane 3 is Ataxin-2 (E3B3Z) Rabbit mAb. Western blot analysis was performed using Ataxin-2 (E3B3Z) Rabbit mAb. Anti-rabbit IgG, HRP-linked Antibody #7074 was used as a secondary antibody.