{"product_id":"cst-23187sf","title":"CST,  23187SF, Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody (BSA and Azide Free)","description":"Monoclonal Antibody for studying ataxin-2. Validated for Western Blotting. Highly specific and rigorously validated in-house, Ataxin-2 (E3B3Z) Rabbit Monoclonal Antibody (BSA and Azide Free) (CST #23187) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nThis 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\u0026amp;RUN or CUT\u0026amp;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.\n\u003cb\u003eFormulation\u003c\/b\u003e\nSupplied in 1X PBS (10 mM Na 2 HPO 4 , 3 mM KCl, 2 mM KH 2 PO 4 , and 140 mM NaCl (pH 7.8)). BSA and Azide Free. For standard formulation of this product see product # 35121\n\u003cb\u003eStorage\u003c\/b\u003e\nStore 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.\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nAtaxin-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.\nSpecies Reactivity: Human, Mouse, Rat, Monkey\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val1055 of human ataxin-2 protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nSpinocerebellar 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).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nataxin 2; Ataxin-2; ATX2; ATXN2; FLJ46772; SCA2; Spinocerebellar ataxia type 2 protein; TNRC13; trinucleotide repeat containing 13; Trinucleotide repeat-containing gene 13 protein\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H M R Mk\nSENSITIVITY: Endogenous\nMW (kDa): 150\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46797664878761,"sku":"23187SF","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-23187sf","provider":"Iright","version":"1.0","type":"link"}