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 (68) 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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Showing 9 out of 12 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Escherichia coli (E. coli) Human Unkonjugiert Figure annotation denotes ug of protein loaded and % gel used. 50 μg Anmelden zum Anzeigen 6 bis 8 Tage
HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Anmelden zum Anzeigen 10 bis 12 Tage
Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 11 bis 12 Tage
Hefe Ratte His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
Hefe Huhn His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
Escherichia coli (E. coli) Human His tag   50 μg Anmelden zum Anzeigen 5 bis 8 Tage
Human Unkonjugiert   1 mg Anmelden zum Anzeigen 6 Days

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. Data suggest ATXN3 binds with low-micromolar affinity to both wild-type p97/VCP (zeige vcp Proteine) and mutants linked to proteostasis deficiency multisystem proteinopathy 1 (MSP1; also called hereditary inclusion body myopathy); stoichiometry of binding is one ATXN3 molecule per p97/VCP (zeige vcp Proteine) hexamer in presence of ATP; MSP1 mutants of p97/VCP (zeige vcp Proteine) bind ATXN3 irrespective of nucleotide state. (VCP (zeige vcp Proteine) = valosin-containing protein (zeige vcp Proteine)/ATPase; ATXN3 = ataxin-3)

  2. DNA methylation (zeige HELLS Proteine) levels in the ATXN3 promoter were significantly higher in SCA3/MJD patients

  3. data elucidate the important role of ataxin-3 proteolysis in the pathogenesis of Machado-Joseph disease.

  4. 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.

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

  6. 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.

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

  8. 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.

  9. 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

  10. Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models.

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-signaling in mouse model spinocerebellar ataxia 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 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 L homeolog (atxn3.L)
  • 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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