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ATXN3

  • Official Full Name

    ataxin 3

  • Overview

    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.
  • Synonyms

    ATXN3; ataxin 3; Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) , MJD, SCA3; ataxin-3; ATX3; JOS; josephin; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; olivopontocerebellar;

  • Recombinant Proteins
  • Cell & Tissue Lysates
  • Protein Pre-coupled Magnetic Beads
  • Chicken
  • Human
  • Mouse
  • Rat
  • Zebrafish
  • E. coli
  • E.coli
  • E.Coli or Yeast
  • HEK293
  • HEK293T
  • In Vitro Cell Free System
  • Mammalian Cell
  • Mammalian cells
  • Sf9 Insect Cell
  • Wheat Germ
  • C
  • His
  • DDK
  • Flag
  • GST
  • His (Fc)
  • Avi
  • Myc
  • MYC
  • Myc|DDK
  • N/A
  • No tag
Species Cat.# Product name Source (Host) Tag Protein Length Price
Human ATXN3-173H Active Recombinant Human ATXN3, GST-tagged E.coli GST
Human ATXN3-1032H Recombinant Human ATXN3 protein, GST-tagged Wheat Germ GST
Human ATXN3-478H Recombinant Human Ataxin 3 E.coli N/A
Human ATXN3-739H Recombinant Human ATXN3 Protein, DDK-tagged Sf9 Insect Cell DDK
Human ATXN3-27045TH Recombinant Human ATXN3 E.coli N/A
Human ATXN3-52HCL Recombinant Human ATXN3 lysate HEK293 N/A
Human ATXN3-3036H Recombinant Human ATXN3 Protein, MYC/DDK-tagged HEK293 Myc/DDK
Human ATXN3-410H Recombinant Human ATXN3 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Human ATXN3-6246H Recombinant Human ATXN3 Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
Human ATXN3-1896HFL Recombinant Full Length Human ATXN3 Protein, C-Flag-tagged Mammalian cells Flag
Human ATXN3-1053HF Recombinant Full Length Human ATXN3 Protein, GST-tagged In Vitro Cell Free System GST 79 amino acids
Human ATXN3-0368H Recombinant Human ATXN3 Protein (E2-K361), His tagged E.coli His E2-K361
Human ATXN3-0367H Recombinant Human ATXN3 Protein (E2-K361), Tag Free E.coli No tag E2-K361
Human ATXN3-2786H Recombinant Human ATXN3 protein(11-290 aa), C-His-tagged E. coli C-His 11-290 aa
Human ATXN3-410H-B Recombinant Human ATXN3 Protein Pre-coupled Magnetic Beads HEK293
Mouse Atxn3-478M Recombinant Mouse Atxn3 Protein, MYC/DDK-tagged HEK293T MYC/DDK
Rat Atxn3-3689R Recombinant Rat Atxn3, His-tagged E.Coli or Yeast His 355
Rat ATXN3-902R Recombinant Rat ATXN3 Protein Mammalian Cell His
Rat ATXN3-558R Recombinant Rat ATXN3 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Rat ATXN3-558R-B Recombinant Rat ATXN3 Protein Pre-coupled Magnetic Beads HEK293
Zebrafish ATXN3-11326Z Recombinant Zebrafish ATXN3 Mammalian Cell His
Chicken ATXN3-5928C Recombinant Chicken ATXN3 Mammalian Cell His
  • Involved Pathway
  • Protein Function
  • Interacting Protein

ATXN3 involved in several pathways and played different roles in them. We selected most pathways ATXN3 participated on our site, such as Protein processing in endoplasmic reticulum, which may be useful for your reference. Also, other proteins which involved in the same pathway with ATXN3 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name Pathway Related Protein
Protein processing in endoplasmic reticulumUBE2D1B;P4HB;HSP90AB1;CUL1B;DNAJC1;CRYAA;CRYAB;SEC13;NPLOC4

ATXN3 has several biochemical functions, for example, ATPase binding, Lys48-specific deubiquitinase activity, Lys63-specific deubiquitinase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by ATXN3 itself. We selected most functions ATXN3 had, and list some proteins which have the same functions with ATXN3. You can find most of the proteins on our site.

Function Related Protein
ATPase bindingATP1B3;RAB4A;CHMP4A;ATP6V0A2B;ATPIF1;ATP6V0A1;PIH1D1;ALDOB;RALB
Lys48-specific deubiquitinase activityOTUD7B;ATXN3;YOD1
Lys63-specific deubiquitinase activityCYLD;YOD1;ATXN3
identical protein bindingPCNA;APLP2;TTR;SCAND1;TRIM8;CLDN11;ORAI1;CLDN2;UGP2
omega peptidase activityGGH;UCHL1;JOSD2;USP5;USP13;ATXN3L;UCHL5;APEH;ATXN3
protein bindingNGFR;MAP2K4;HGS;TXLNA;TRAF1;HLTF;NOA1;PHF8;MAP2K6
ubiquitin protein ligase bindingUBE2H;FZD6;TRIM37;SKI;UBXN6;ASB4;YWHAZ;FBXW7;UBE2V1
ubiquitin-specific protease activityUSP6;USP40;UCHL3;USP17L5;USP47;USP20;USP18;OTUD7B;OTULIN

ATXN3 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with ATXN3 here. Most of them are supplied by our site. Hope this information will be useful for your research of ATXN3.

VCP; UBC; RAD23B

  • Q&As
  • Reviews

Q&As (6)

Ask a question
What are the clinical manifestations of SCA3? 10/15/2022

The clinical manifestations of SCA3 include ataxia (especially limb motor coordination disorder), myoclonus, ocular muscle movement abnormalities, extrapyramidal symptoms, dysphagia, language disorder, etc. As the disease progresses, patients have more and more significant difficulties with walking, balance, and other movements.

Which cellular processes are ATXN3 proteins involved in? 02/23/2022

ATXN3 protein is involved in many important cellular processes, including protein degradation, cell signaling, apoptosis, RNA metabolism, etc. It plays an important role in the regulation of cellular functions.

Is there any treatment for SCA3? 07/09/2021

At present, there is no cure for SCA3, but some adjuvant therapies can be used to improve symptoms. Physical therapy, speech therapy and swallowing function training can improve the quality of life of patients. In addition, there are some studies on protein degradation pathways for abnormal aggregation of ATXN3 protein, but they are still in the experimental stage.

Is ATXN3 protein associated with other diseases? 03/23/2021

In addition to its association with SCA3, ATXN3 protein may also be associated with some other diseases. For example, studies have shown that ATXN3 protein is related to the occurrence and development of neurodegenerative diseases such as Parkinson's disease and Huntington's disease, but the specific mechanism needs to be further studied and verified.

How do ATXN3 protein abnormalities cause SCA3? 07/06/2020

SCA3 is caused by mutations in the ATXN3 gene that lead to excessive repeat of polyglutamine (PolyQ) sequences in ATXN3 protein. This abnormal expansion will cause abnormal ATXN3 protein folding and aggregation, leading to impaired cell function and eventually triggering SCA3 symptoms.

Which disease is ATXN3 protein associated with? 11/26/2019

ATXN3 protein is associated with autodominant genetic disease Spinocerebellar Ataxia Type 3 (SCA3). SCA3 is a neurodegenerative disease characterized by ataxia, extrapyramidal symptoms, and motor neuron dysfunction.

Customer Reviews (3)

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Reviews
06/21/2021

    ATXN3 is very catalytic active and shows excellent catalytic performance even at low concentrations.

    08/14/2019

      ATXN3 detection method is sensitive and can detect trace amounts of protein, which is well suited for our study.

      03/25/2019

        Using ATXN3, the reproducibility of my experiments is very good, which facilitates subsequent data analysis and experimental manipulation.

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