Product Name:

Ataxin 1


Product Number:

ab-nn205-1

Price:

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Target Full Name: Ataxin-1

Target Alias: Ataxin-1; ATX1; Atxn1; D6S504E; OTTHUMP00000016065; SCA1; Spinocerebellar ataxia type 1 protein homolog

Product Type Specific: Ataxin 1 pan-specific antibody

Antibody Code: NN205-1

Antibody Target Type: Pan-specific

Protein UniProt: P54253

Protein SigNET: Ataxin 1

Antibody Type: Monoclonal

Antibody Host Species: Mouse

Antibody Ig Isotype Clone: IgG2b

Antibody Immunogen Source: Synthetic peptide based mouse Ataxin-1 (Uniprot ID P54254)

Antibody Immunogen Sequence: ATTPSQRSQLEAYSTLLANMGSLSQAPGHKVEPP

Antibody Immunogen Description: Corresponds to amino acid residues A164-P197. Rat: 100% identity (34/34 amino acids identical). Human: 88% identity (30/34 amino acids identical).

Production Method: Protein G purified

Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.

Antibody Concentration: 1 mg/ml

Storage Buffer: Phosphate buffered saline pH 7.4, 50% glycerol, 0.1% sodium azide

Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.

Product Use: Western blotting | Immunohistochemistry | Immunoprecipitation

Antibody Dilution Recommended: WB (1:1000); optimal dilutions for assays should be determined by the user.

Antibody Potency: In mouse brain lysates, this antibody detects a ~85 kDa protein in cell and tissue lysates by Western blotting.

Antibody Species Reactivity: This antibody detects the target protein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Dog | Rat | Mouse.

Antibody Positive Control: 1 µg/ml of SMC-455 was sufficient for detection of Ataxin-1 in 20 µg of rat brain lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody.

Antibody Specificity: Very high

Scientific Background: Ataxin-1 is a member of the ATXN1 protein family and contains a single AXH domain. Ataxin-1 is a primarily nuclear protein that acts as a DNA-binding transcriptional repressor involved in gene regulation and RNA processing. It binds to DNA and acts as a transcriptional corepressor, regulating genes. It also binds RNA and is involved in protein production/processing. It interacts with proteins like SMRT (silencing mediator of retinoid and thyroid hormone receptors) and Capicua (CIC). It is a phosphorylated (at positions such as Ser-776) and sumoylated protein. It contains an AXH domain (ataxin-1 and HMG-box protein 1) that mediates interactions with other proteins. Mutant forms of Ataxin-1 with expanded polyglutamine (polyQ) tracts (pathological, usually >40) cause spinocerebellar ataxia type-1 (SCA1), which is a progressive, neurodegenerative disease that is autosomal dominant and primarily affects the Purjinke cells found in brain stem neuronal populations and the cerebellum. Mutant ataxin-1 escapes normal degradation systems (e.g., E3-ligase CHIP), causing a toxic gain-of-function that results in cerebellum degeneration. Expression of Ataxin-1 is almost ubiquitous, except in the brain where it is isolated to populations of neurons. This description may include information annotated by UniProt and/or Google AI.