CST, 35121S, Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying ataxin-2. Validated for Western Blotting,Immunoprecipitation. Available in 2 sizes. Highly specific and rigorously validated in-house, Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody (CST #35121) is ready to ship. Product Usage Information 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. For a carrier free (BSA and azide free) version of this product see product # 23187 . Protocol Available protocols: Western Blotting, Immunoprecipitation Specificity / Sensitivity Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody 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 gene, which encodes the protein ataxin-2 (2). Intermediate-length polyQ repeats in 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). 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 Specification REACTIVITY: H M R Mk SENSITIVITY: Endogenous MW (kDa): 150 Source/Isotype: Rabbit IgG