ATXN3
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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
- Human
- Zebrafish
- Rat
- Chicken
- Mouse
- E.coli
- Wheat Germ
- Mammalian Cell
- E.Coli or Yeast
- Sf9 Insect Cell
- HEK293
- In Vitro Cell Free System
- Mammalian cells
- HEK293T
- GST
- His
- Non
- Flag
- Myc&DDK
- His&Fc&Avi
Involved Pathway
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 reticulum | UBE2E1,UBE2E3,LMAN1L,DNAJB12A,CUL1A,FBXO2,XBP1,DNAJC3,P4HB,SSR1 |
Protein Function
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 |
|---|---|
| omega peptidase activity | USP13,UCHL5,ATXN3L,UCHL1,JOSD2,APEH,GGH,USP5,JOSD1 |
| Lys63-specific deubiquitinase activity | YOD1,CYLD |
| Lys48-specific deubiquitinase activity | YOD1,OTUD7B |
| protein binding | MRPL23,STX11,NKD1,LAIR1,LOXL3,GLP1R,APPBP2,ST6GAL1,GDPD5,CELF1 |
| ubiquitin protein ligase binding | TMBIM6,FLJ11011L,CALR,NPLOC4,SUMO1,MUL1,SMAD6,CUL9,HSPA1B,FANCL |
| ubiquitin-specific protease activity | USP24,STAMBP,USP19,ZRANB1,COPS5,USP25,USP46,OTUD6A,USP47,USP9X |
| ATPase binding | CHMP4A,ABCA1,PGR,PDE4D,CAV1,TOR1AIP1,DNAJB1,DERL1,ATP6V0A1B,LCK |
| identical protein binding | ZNF703,RAF1,SH2B2,AHI1,SMN2,CRYAA,MUL1,BRI3,UGP2,BCL2L1 |
Interacting Protein
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
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