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Target antigen
Ataxin 3
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Clonality
Polyclonal antibody
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Clone
Polyclonal antibody
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Raised in
rabbit
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Type of the antibody
IgG polyclonal antibody
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Product form
freeze-dried
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Reacts with species
human Predicted to work with: mouse, rat
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Analyses
WB,IHC-P
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Immunogen
A synthetic peptide corresponding to a sequence at the N-terminus of human Ataxin 3(7-21aa EKQEGSLCAQHCLNN), identical to the related rat and mouse sequences.
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Product configuration
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
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Purification
Immunogen affinity purified.
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Solubilization
The powdered antibody should be dissolved in 0.2 ml of distilled water to achieve final concentration of 500ug/ml
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Storage condtions
Keep the Ataxin 3 Antibodyat minus twenty degrees Celsius for 1 year. The ready-to-use solutions can be stored at four degrees Celsius for a month. Our specialsits recommend to freeze the aliquotes at minus twenty degrees Celsius for long-term application. Multiple procedures of freezing and thawing influence the specifity and reactivity of the antibody in a negative way.
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Tips
The Ataxin 3 Antibody did not cross-reacted with other proteins during the test procedures. This antobdy is intended to be used for research analyses and it is not applicale for in vitro diagnostics.
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Background
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 range 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.
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Related articles
1. Burnett, B., Li, F., Pittman, R. N. The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum. Molec. Genet. 12: 3195-3205, 2003. 2. Paulson, H. L., Das, S. S., Crino, P. B., Perez, M. K., Patel, S. C., Gotsdiner, D., Fischbeck, K. H., Pittman, R. N.Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.Ann. Neurol. 41: 453-462, 1997.
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Gene Name
ATXN3
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Protein Name
Ataxin-3
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Gene Full Name
ataxin 3
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Synonyms
AT3 antibody|Ataxin 3 antibody|Ataxin-3 antibody|ATX3 antibody|ATX3_HUMAN antibody|ATXN3 antibody|EC 3.4.22. antibody|JOS antibody|Josephin antibody|Machado Joseph disease(spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) antibody|Machado Joseph disease antibody|Machado Joseph disease protein 1 antibody|Machado-Joseph disease protein 1 antibody|Machado-Joseph disease protein 1 homolog antibody|MJD antibody|MJD gene antibody|MJD1 antibody|Olivopontocerebellar ataxia 3 antibody|OTTHUMP00000221583 antibody|OTTHUMP00000221585 antibody|OTTHUMP00000221586 antibody|OTTHUMP00000221587 antibody|OTTHUMP00000231995 antibody|OTTHUMP00000231997 antibody|Rsca3 antibody|SCA3 antibody|SCA3 gene antibody|Spinocerebellar ataxia type 3 protein antibody
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Uniprot ID
P54252
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Properties
If you buy Antibodies supplied by boster they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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French translation
anticorps
