Ataxin 1 Antibody

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.

Detects ~85kDa. 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.

Ataxin-1 Antibody, ATX1 Antibody, Atxn1 Antibody, D6S504E Antibody, OTTHUMP00000016065 Antibody, SCA1 Antibody, Spinocerebellar ataxia type 1 protein Antibody, Spinocerebellar ataxia type 1 protein homolog Antibody

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).

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.