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Render Timestamp: 2024-12-20T12:06:05.788Z
Commit: f2d32940205a64f990b886d724ccee2c9935daff
XML generation date: 2024-09-30 01:59:42.389
Product last modified at: 2024-11-27T22:00:09.085Z
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PDP - Template Name: Monoclonal Antibody
PDP - Template ID: *******c5e4b77
  1. Products /
  2. Primary Antibodies /
  3. Ataxin-2 (E3B3Z) Rabbit mAb
R Recombinant
Recombinant: Superior lot-to-lot consistency, continuous supply, and animal-free manufacturing.

Ataxin-2 (E3B3Z) Rabbit mAb #35121

Filter:
  • WB
  • IP

    Supporting Data

    REACTIVITY H M R Mk
    SENSITIVITY Endogenous
    MW (kDa) 150
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    • IP-Immunoprecipitation 
    Species Cross-Reactivity Key:
    • H-Human 
    • M-Mouse 
    • R-Rat 
    • Mk-Monkey 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000
    Immunoprecipitation 1:50

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    Protocol

    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.

    Species Reactivity:

    Human, Mouse, Rat, Monkey

    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).
    1. Magaña, J.J. et al. (2013) Mol Neurobiol 47, 90-104.
    2. Laffita-Mesa, J.M. et al. (2021) Curr Opin Neurol 34, 578-588.
    3. Elden, A.C. et al. (2010) Nature 466, 1069-75.
    4. Sproviero, W. et al. (2017) Neurobiol Aging 51, 178.e1-178.e9.
    5. Glass, J.D. et al. (2022) Brain 145, 2671-2676.
    6. Yokoshi, M. et al. (2014) Mol Cell 55, 186-98.
    7. Inagaki, H. et al. (2020) J Biol Chem 295, 15810-15825.
    8. Ralser, M. et al. (2005) J Mol Biol 346, 203-14.
    9. Nonhoff, U. et al. (2007) Mol Biol Cell 18, 1385-96.
    10. Bakthavachalu, B. et al. (2018) Neuron 98, 754-766.e4.
    11. Neumann, M. et al. (2006) Science 314, 130-3.
    12. Liu-Yesucevitz, L. et al. (2010) PLoS One 5, e13250.
    13. Hart, M.P. and Gitler, A.D. (2012) J Neurosci 32, 9133-42.
    14. Watanabe, R. et al. (2020) Acta Neuropathol Commun 8, 176.
    15. Ostrowski, L.A. et al. (2017) Genes (Basel) 8, 157. doi: 10.3390/genes8060157.
    16. Scoles, D.R. et al. (2017) Nature 544, 362-366.
    17. Becker, L.A. et al. (2017) Nature 544, 367-371.

    Database Links

    UniProt ID: Q99700


    Entrez-Gene Id: 6311


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