ATXN3 Antibody

Mfr.No.	R32137
Clonality	Polyclonal
Description	ATXN3 (Ataxin 3), also known as AT3, MJD GENE, MJD1, SCA3 GENE, ATX3, JOS, Spinocerebellar ataxia-3, Machado-Joseph disease protein 1, is a protein that in humans is encoded by the ATXN3 gene. ATXN3 ranges in size from 360 to 374 amino acids. Using Northern blot analysis showed that ATXN3 mRNA was ubiquitously expressed in human tissues. They detected at least 4 ATXN3 transcripts of 1.4, 1.8, 4.5, and 7.5 kb and suggested that the different mRNA species probably result from differential splicing and polyadenylation. Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by the ATXN3 gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is an inverse correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. Ataxin-3 interacted with 2 human homologs of the yeast DNA repair protein RAD23, HHR23A (RAD23A) and HHR23B (RAD23B). Both normal and mutant ataxin-3 proteins interacted with the ubiquitin-like domain at the N terminus of the HHR23 proteins, which is a motif important for nucleotide excision repair. However, in HEK 293 cells, HHR23A was recruited to intranuclear inclusions formed by the mutant ataxin-3 through its interaction with ataxin-3.
Formulation	0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host	Rabbit
Immunogen Region	Amino acids EEDLQRALALSRQEIDMEDEEADLRRAIQ of human Ataxin 3 were used as the immunogen for the ATXN3 antibody.
Isotype	IgG
Predicted Reactivity	Human, Rat
Reactivity	Rat, Human
Recombinant	No
Subcellular Location	Nuclear and cytoplasmic

Antigen	Ataxin 3
Uniprot	P54252

Format	Antigen affinity purified
Purification	Antigen affinity
Storage	After reconstitution, the ATXN3 antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Applications	IHC-P, IF, WB, FACS
Dilution	Western blot: 0.1-0.5ug/ml,Immunohistochemistry (FFPE): 0.5-1ug/ml,Flow cytometry: 1-3ug/million cells,Immunofluorescence: 2-4ug/ml