Product Name: Ataxin 1
Product Number: AB-NN205-2
Size: 25 µg      Price:89.00
      $US
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: P54253

Antibody Type: Monoclonal

Antibody Host Species: Mouse

Antibody Ig Isotype Clone: IgG1

Antibody Immunogen Source: Synthetic peptide amino acids 746-761 (RKRRWSAPETRKLEKS) of mouse ataxin-1. 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: Human | Mouse | Rat

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 pan-specific antibody (Cat. No.: AB-NN205-1)

Scientific Background: Ataxin-1 is a member of the ATXN1 protein family and contains a single AXH domain. It is a neurodegenerative disorder protein thought to have a role in the metabolism of RNA as it has been shown to localize to the RNA and transcription dependent inclusions within the nucleus. A mutation of Ataxin-1 is the cause of spinocerebellar ataxia type-1 (SCA1), a progressive, neurodegenerative disease that is autosomal dominant and primarily affects the Purjinke cells found in brain stem neuronal populations and the cerebellum. Expression of Ataxin-1 is almost ubiquitous, except in the brain where it is isolated to populations of neurons.