Ataxin 3 Proteine (ATXN3)

Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. Zusätzlich bieten wir Ihnen Ataxin 3 Antikörper (67) und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
ATXN3 4287 P54252
ATXN3 110616 Q9CVD2
ATXN3 60331 O35815
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Top Ataxin 3 Proteine auf

Showing 9 out of 14 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
HOST_Escherichia coli (E. coli) Human Unkonjugiert Figure annotation denotes ug of protein loaded and % gel used. 50 μg Anmelden zum Anzeigen 10 bis 13 Tage
356,80 €
Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 46 Days
5.173,33 €
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 46 Days
5.173,33 €
HOST_HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Anmelden zum Anzeigen 6 bis 8 Tage
748,00 €
HOST_Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 7 bis 8 Tage
345,60 €
Hefe Ratte His tag   1 mg Anmelden zum Anzeigen 58 bis 70 Tage
2.826,83 €
Hefe Huhn His tag   1 mg Anmelden zum Anzeigen 58 bis 70 Tage
2.849,52 €
HOST_Escherichia coli (E. coli) Human His tag   50 μg Anmelden zum Anzeigen 2 bis 5 Tage
254,00 €
Human Unkonjugiert   2 μg Anmelden zum Anzeigen 10 bis 11 Tage
186,70 €

ATXN3 Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Human , , ,
, ,
Mouse (Murine)

Rat (Rattus)

Weitere Proteine zu Ataxin 3 (ATXN3) Interaktionspartnern

Human Ataxin 3 (ATXN3) Interaktionspartner

  1. The findings reveal ATXN3 to be a novel deubiquitinase of Chk1 (zeige CHEK1 Proteine), providing a new mechanism of Chk1 (zeige CHEK1 Proteine) stabilization in genome integrity maintenance.

  2. Segregation patterns and factors influencing instability of expanded ATXN3 CAG transmissions in Machado-Joseph disease have been analyzed.

  3. Our data reveal a previously unrecognized balance between pathogenic and potentially therapeutic properties of the ataxin-3-Rad23 interaction; they highlight this interaction as critical for the toxicity of the SCA3 protein, and emphasize the importance of considering protein context when pursuing suppressive avenues.

  4. the opposing activities of RNF4 and ataxin-3 consolidate robust MDC1-dependent signaling and repair ofDNA double-strand break.

  5. we demonstrated that neural differentiation in these iPS (zeige SLC27A4 Proteine) cells was accompanied by autophagy and that rapamycin promoted autophagy through degradation of mutant ATXN3 proteins in neurally differentiated spinocerebellar ataxia-3 (zeige SCN8A Proteine) human induced pluripotent stem cells (p < 0.05). In conclusion, patient-derived iPS (zeige SLC27A4 Proteine) cells are a good model for studying the mechanisms of SCA3 and may provide a tool for drug discovery in vitro.

  6. 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

  7. Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia (zeige USP14 Proteine) type 3 models.

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

  9. 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 (zeige AR Proteine) AEs (zeige AES Proteine)>/=50

  10. 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.

Mouse (Murine) Ataxin 3 (ATXN3) Interaktionspartner

  1. findings identify a novel molecular link between ATX-3 and p53 (zeige TP53 Proteine)-mediated cell death and provide an explanation for the direct involvement of p53 (zeige TP53 Proteine) in SCA3 disease pathogenesis

  2. 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 (zeige HSPA5 Proteine) protein levels at 30 weeks of age, one of its known neuroprotective effects

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

  4. 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

  5. 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.

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

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

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

  9. Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia (zeige USP14 Proteine) Type 3 mice.

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

Ataxin 3 (ATXN3) Protein Überblick

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit Ataxin 3 Proteine (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

Bezeichner auf Proteinebene für Ataxin 3 Proteine (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

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
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