Product Name:
Ataxin 1
Product Number:
ab-nn205-2
Target Full Name: Spinocerebellar ataxia type 1 protein
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: NN205-2
Antibody Target Type: Pan-specific
Protein UniProt: P54253
Protein SigNET: Ataxin 1
Antibody Type: Monoclonal
Antibody Host Species: Mouse
Antibody Ig Isotype Clone: IgG1
Antibody Immunogen Source: Synthetic peptide patterned after mouse Ataxin-1 (Uniprot ID P54254)
Antibody Immunogen Sequence: RKRRWSAPETRKLEKS
Antibody Immunogen Description: Corresponds to amino acid residues R746-S761. Rat: 93% identity (15/16 amino acids identical). Human: 87% identity (14/16 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 | ICC/Immunofluorescence
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-475 was sufficient for detection of Ataxin 1 in 20 µg of COS cells transiently transfected with Ataxin-1 lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody.
Antibody Specificity: Very high
Antibody Cross Reactivity: No cross-reactivity against phosphor S751-Ataxin-1. Minimal cross-reactivity against S751A mutant of Ataxin-1 by ELISA and immunofluorescence and negative by immunoblot.
Related Product 1: Ataxin 1 expression antibody (Cat. No.: AB-NN205-1)
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.