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Ataxin 3 Proteins (ATXN3)

Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. Additionally we are shipping Ataxin 3 Antibodies (100) and Ataxin 3 Kits (4) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
ATXN3 4287 P54252
ATXN3 110616 Q9CVD2
ATXN3 60331 O35815
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Top Ataxin 3 Proteins at antibodies-online.com

Showing 9 out of 15 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human Un-conjugated Figure annotation denotes ug of protein loaded and % gel used. 50 μg Log in to see 7 to 9 Days
$364.64
Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Rat His tag   1 mg Log in to see 56 to 66 Days
$2,968.17
Details
Yeast Chicken His tag   1 mg Log in to see 56 to 66 Days
$2,992.00
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated   2 μg Log in to see 6 to 11 Days
$142.86
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details
Human Un-conjugated   2 μg Log in to see 6 Days
$168.54
Details

ATXN3 Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Mouse (Murine)

Rat (Rattus)

More Proteins for Ataxin 3 (ATXN3) Interaction Partners

Human Ataxin 3 (ATXN3) interaction partners

  1. 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, CACNA1A, ATXN3, and ATXN7 genes

  2. Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia (show USP14 Proteins) type 3 models.

  3. USP19_b up-regulates the protein levels of the polyglutamine (polyQ)-containing proteins, ataxin-3 (Atx3) and huntingtin (Htt (show HTT Proteins)), and thus promotes aggregation of their polyQ-expanded species in cell models

  4. 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: ANsAIs (show AR Proteins) AEs (show AES Proteins)>/=50

  5. 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.

  6. Data show that homozygosity for Machado-Joseph disease (MJD)/SCA3 protein enhances the clinical severity of the disease.

  7. A multistage aggregation mechanism for ataxin-3 is described in which flanking domain self-assembly precedes polyglutamine aggregation yet is influenced by polyglutamine expansion.

  8. 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.

  9. 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.

  10. polyglutamine expansion increases the molecular mobility of two juxtaposed helices critical to ataxin-3 deubiquitinase activity

Mouse (Murine) Ataxin 3 (ATXN3) interaction partners

  1. 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 Proteins) protein levels at 30 weeks of age, one of its known neuroprotective effects

  2. work suggests that in Machado-Joseph disease, mutant ataxin-3 drives an abnormal reduction of ataxin-2 (show ATXN2 Proteins) levels, which overactivates poly(A)-binding protein, increases translation of mutant ataxin-3 and other proteins and aggravates Machado-Joseph disease.

  3. 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

  4. 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.

  5. While ataxin-3 may participate in protein quality control pathways, it does not critically regulate the handling of mutant htt (show HTT Proteins) or contribute to major features of disease pathogenesis in Huntington disease (show HTT Proteins).

  6. Results suggest that postnatal nuclear accumulation of mutant ataxin-3 disrupts dendritic differentiation and mGluR (show GRM8 Proteins)-signaling in mouse model spinocerebellar ataxia (show USP14 Proteins) type 3 Purkinje cells

  7. Lentiviral-based expression of mutant atxn-3 in the mouse cerebellum induces localized neuropathology sufficient to generate a behavioral ataxic phenotype.

  8. Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia (show USP14 Proteins) Type 3 mice.

  9. the efficacy of gene silencing in blocking the MJD-associated motor-behavior and neuropathological abnormalities

  10. the sequestration of misfolded SOD1 (show SOD1 Proteins) into aggresomes, which is driven by ataxin-3, plays an important role in attenuating protein misfolding-induced cell toxicity.

Ataxin 3 (ATXN3) Protein Profile

Protein Summary

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.

Gene names and symbols associated with Ataxin 3 Proteins (ATXN3)

  • ataxin 3 (atxn3)
  • ataxin 3 (ATXN3)
  • ataxin-3 (MICPUN_104046)
  • ataxin-3 (MICPUCDRAFT_46658)
  • ataxin 3 (Atxn3)
  • 2210008M02Rik protein
  • AI463012 protein
  • AI647473 protein
  • AT3 protein
  • ataxin-3 protein
  • ATX3 protein
  • ATXN3 protein
  • JOS protein
  • MGC83584 protein
  • MJD protein
  • MJD1 protein
  • Rsca3 protein
  • Sca3 protein
  • zgc:56323 protein

Protein level used designations for Ataxin 3 Proteins (ATXN3)

ataxin-3 , ataxin 3 , ataxin-3-like , 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 , josephin , 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

GENE ID SPECIES
394079 Danio rerio
480229 Canis lupus familiaris
549143 Xenopus (Silurana) tropicalis
738729 Pan troglodytes
790880 Bos taurus
100144422 Sus scrofa
100171397 Ovis aries
444085 Xenopus laevis
100393557 Callithrix jacchus
8249057 Micromonas sp. RCC299
9682664 Micromonas pusilla CCMP1545
4287 Homo sapiens
110616 Mus musculus
60331 Rattus norvegicus
378424 Gallus gallus
Selected quality suppliers for Ataxin 3 Proteins (ATXN3)
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