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  3. Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody (BSA and Azide Free)
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Recombinant: Superior lot-to-lot consistency, continuous supply, and animal-free manufacturing.

Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody (BSA and Azide Free) #23187

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  • WB

    Product Specifications

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

    Product Information

    Product Usage Information

    This product is the carrier free version of product #35121. All data were generated using the same antibody clone in the standard formulation which contains BSA and glycerol.

    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.

    BSA and Azide Free antibodies are quality control tested by size exclusion chromatography (SEC) to determine antibody integrity.

    Formulation

    Supplied in 1X PBS (10 mM Na2HPO4, 3 mM KCl, 2 mM KH2PO4, and 140 mM NaCl (pH 7.8)). BSA and Azide Free.

    For standard formulation of this product see product #35121

    Storage

    Store at -20°C. This product will freeze at -20°C so it is recommended to aliquot into single-use vials to avoid multiple freeze/thaw cycles. A slight precipitate may be present and can be dissolved by gently vortexing. This will not interfere with antibody performance.

    Specificity / Sensitivity

    Ataxin-2 (E3B3Z) Rabbit mAb (BSA and Azide Free) 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.

    Alternate Names

    ataxin 2; Ataxin-2; ATX2; ATXN2; FLJ46772; SCA2; Spinocerebellar ataxia type 2 protein; TNRC13; trinucleotide repeat containing 13; Trinucleotide repeat-containing gene 13 protein

    Database Links

    UniProt ID: Q99700


    Entrez-Gene Id: 6311


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    For Research Use Only. Not for Use in Diagnostic Procedures.
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