Revision 1

#35121

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Orders:

877-616-CELL (2355)

[email protected]

Support:

877-678-TECH (8324)

3 Trask Lane | Danvers | Massachusetts | 01923 | USA

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

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.

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).

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

Trademarks and Patents

Cell Signaling Technology is a trademark of Cell Signaling Technology, Inc.

All other trademarks are the property of their respective owners. Visit cellsignal.com/trademarks for more information.

Limited Uses

Except as otherwise expressly agreed in a writing signed by a legally authorized representative of CST, the following terms apply to Products provided by CST, its affiliates or its distributors. Any Customer's terms and conditions that are in addition to, or different from, those contained herein, unless separately accepted in writing by a legally authorized representative of CST, are rejected and are of no force or effect.

Products are labeled with For Research Use Only or a similar labeling statement and have not been approved, cleared, or licensed by the FDA or other regulatory foreign or domestic entity, for any purpose. Customer shall not use any Product for any diagnostic or therapeutic purpose, or otherwise in any manner that conflicts with its labeling statement. Products sold or licensed by CST are provided for Customer as the end-user and solely for research and development uses. Any use of Product for diagnostic, prophylactic or therapeutic purposes, or any purchase of Product for resale (alone or as a component) or other commercial purpose, requires a separate license from CST. Customer shall (a) not sell, license, loan, donate or otherwise transfer or make available any Product to any third party, whether alone or in combination with other materials, or use the Products to manufacture any commercial products, (b) not copy, modify, reverse engineer, decompile, disassemble or otherwise attempt to discover the underlying structure or technology of the Products, or use the Products for the purpose of developing any products or services that would compete with CST products or services, (c) not alter or remove from the Products any trademarks, trade names, logos, patent or copyright notices or markings, (d) use the Products solely in accordance with CST Product Terms of Sale and any applicable documentation, and (e) comply with any license, terms of service or similar agreement with respect to any third party products or services used by Customer in connection with the Products.

Orders: 877-616-CELL (2355) [email protected] Support: 877-678-TECH (8324) [email protected] Web: cellsignal.com
For Research Use Only. Not for Use in Diagnostic Procedures.

Revision 1

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Western blot analysis of extracts from various cell lines using Ataxin-2 (E3B3Z) Rabbit mAb.

Western Blotting Image 1: Ataxin-2 (E3B3Z) Rabbit mAb

Western blot analysis of extracts from various cell lines and tissue using Ataxin-2 (E3B3Z) Rabbit mAb.

Western Blotting Image 2: 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.

Immunoprecipitation Image 1: Ataxin-2 (E3B3Z) Rabbit mAb
Orders: 877-616-CELL (2355) [email protected] Support: 877-678-TECH (8324) [email protected] Web: cellsignal.com
For Research Use Only. Not for Use in Diagnostic Procedures.