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Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. Additionally we are shipping Ataxin 3 Proteins (15) and Ataxin 3 Kits (4) and many more products for this protein.
Showing 10 out of 101 products:
Human Polyclonal Ataxin 3 Primary Antibody for EIA, IHC (p) - ABIN357621
Albrecht, Golatta, Wüllner, Lengauer: Structural and functional analysis of ataxin-2 and ataxin-3. in European journal of biochemistry / FEBS 2004
Show all 5 references for ABIN357621
Human Monoclonal Ataxin 3 Primary Antibody for WB - ABIN2666310
Li, Macfarlan, Pittman, Chakravarti: Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities. in The Journal of biological chemistry 2002
Show all 4 references for ABIN2666310
Human Polyclonal Ataxin 3 Primary Antibody for EIA, FACS - ABIN950546
Reina, Zhong, Pittman: Proteotoxic stress increases nuclear localization of ataxin-3. in Human molecular genetics 2009
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Human Monoclonal Ataxin 3 Primary Antibody for EIA, FACS - ABIN781665
Trottier, Cancel, An-Gourfinkel, Lutz, Weber, Brice, Hirsch, Mandel: Heterogeneous intracellular localization and expression of ataxin-3. in Neurobiology of disease 1999
Show all 2 references for ABIN781665
Human Polyclonal Ataxin 3 Primary Antibody for FACS, IHC (p) - ABIN653191
Jung, Xu, Lessing, Bonini: Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3. in Human molecular genetics 2009
Show all 2 references for ABIN653191
South American cohort did not confirm the effect of the four candidate loci as modifier of onset age: mithocondrial A10398G polymorphism and CAGn at RAI1 (show DOM3Z Antibodies), CACNA1A (show CACNA1A Antibodies), ATXN3, and ATXN7 (show ATXN7 Antibodies) genes
Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia (show USP14 Antibodies) type 3 models.
USP19_b up-regulates the protein levels of the polyglutamine (polyQ)-containing proteins, ataxin-3 (Atx3) and huntingtin (Htt (show HTT Antibodies)), and thus promotes aggregation of their polyQ-expanded species in cell models
Based on these data and other related studies, we presumed that de novo mutations of ATXN3 emerging from large ANs are at least one survival mechanisms of mutational ATXN3 and we can redefine the range of CAG repeats as: ANs=44, 45 =AIs (show AR Antibodies) =49 and AEs (show AES Antibodies)>/=50
Results suggest that the aggregation of Josephin proceeds from the monomer state to the formation of spheroidal intermediates with a native structure. Only successively, these intermediates evolve into misfolded aggregates and into the final fibrils.
Data show that homozygosity for Machado-Joseph disease (MJD)/SCA3 protein enhances the clinical severity of the disease.
A multistage aggregation mechanism for ataxin-3 is described in which flanking domain self-assembly precedes polyglutamine aggregation yet is influenced by polyglutamine expansion.
Ubiquitination of ataxin-3 is not necessary for its proteasomal degradation.Ataxin-3 is regulated by ubiquitin-binding site 2 on its N terminus.Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23.
Machado-Joseph disease patients carrying the rs709930 A allele and rs910369 T allele of ATXN3 experienced an earlier age of onset of approximately 2 to 4 years.
polyglutamine expansion increases the molecular mobility of two juxtaposed helices critical to ataxin-3 deubiquitinase activity
We show that chronic VPA treatment did not modify the ATXN3 inclusion load and astrogliosis in affected brain regions However, VPA chronic treatment was able to increase GRP78 (show HSPA5 Antibodies) protein levels at 30 weeks of age, one of its known neuroprotective effects
work suggests that in Machado-Joseph disease, mutant ataxin-3 drives an abnormal reduction of ataxin-2 (show ATXN2 Antibodies) levels, which overactivates poly(A)-binding protein, increases translation of mutant ataxin-3 and other proteins and aggravates Machado-Joseph disease.
SCA3 knockin mice exhibit robust Atxn3 accumulation both in regions known to be affected in human disease; also display altered splicing of the mutant Atxn3 transcript that results in the formation of a previously described alternative ATXN3 transcript
Data support the importance of ATXN3 in neuronal cells and indicate that an expanded polyQ tract leads to a partial loss of the cellular function of ATXN3 that may be relevant to neurodegeneration.
While ataxin-3 may participate in protein quality control pathways, it does not critically regulate the handling of mutant htt (show HTT Antibodies) or contribute to major features of disease pathogenesis in Huntington disease (show HTT Antibodies).
Results suggest that postnatal nuclear accumulation of mutant ataxin-3 disrupts dendritic differentiation and mGluR (show GRM8 Antibodies)-signaling in mouse model spinocerebellar ataxia (show USP14 Antibodies) type 3 Purkinje cells
Lentiviral-based expression of mutant atxn-3 in the mouse cerebellum induces localized neuropathology sufficient to generate a behavioral ataxic phenotype.
Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia (show USP14 Antibodies) Type 3 mice.
the efficacy of gene silencing in blocking the MJD-associated motor-behavior and neuropathological abnormalities
the sequestration of misfolded SOD1 (show SOD1 Antibodies) into aggresomes, which is driven by ataxin-3, plays an important role in attenuating protein misfolding-induced cell toxicity.
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative 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
, Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3)
, Machado-Joseph disease protein 1
, ataxin 3 variant h
, ataxin 3 variant m
, ataxin 3 variant ref
, olivopontocerebellar ataxia 3
, spinocerebellar ataxia type 3 protein
, Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) homolog
, machado-Joseph disease protein 1 homolog