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ATXN3 binds RAD23
Stable Identifier
R-HSA-5688786
Type
Reaction [binding]
Species
Homo sapiens
Compartment
nucleoplasm
ReviewStatus
5/5
Locations in the PathwayBrowser
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Metabolism of proteins (Homo sapiens)
Post-translational protein modification (Homo sapiens)
Deubiquitination (Homo sapiens)
Josephin domain DUBs (Homo sapiens)
ATXN3 binds RAD23 (Homo sapiens)
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Ataxin-3 (ATXN3) is a ubiquitously-expressed deubiqutinating enzyme with important functions in the proteasomal protein degradation pathway and regulation of transcription.
ATXN3 interacts with RAD23A and RAD23B, multiubiquitin chain receptors involved in modulation of proteasomal degradation. RAD23 binds to Lysine-48-linked (K48) polyubiquitin chains, and with a lower affinity to K63-linked chains, in a length-dependent manner. RAD23 is proposed to bind simultaneously to the 26S proteasome and polyubiquitinated substrates, thereby assisting their delivery to the proteasome (Wang et al. 2000).
The C-terminus of ATXN3 contains a polyglutamine (PolyQ) region that, when mutationally expanded to over 52 glutamines, causes the protein to form aggregates that are a hallmark of the neurodegenerative disease spinocerebellar ataxia 3 (SCA3) (Kawaguchi et al. 1994, Evers et al. 2014).
Literature References
PubMed ID
Title
Journal
Year
10915768
Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B
Kanazawa, I
,
Kotliarova, S
,
Wang, G
,
Nukina, N
,
Sawai, N
Hum. Mol. Genet.
2000
Participants
Input
ATXN3 [nucleoplasm]
(Homo sapiens)
RAD23 [nucleoplasm]
(Homo sapiens)
Output
ATXN3:RAD23 [nucleoplasm]
(Homo sapiens)
Participates
as an event of
Josephin domain DUBs (Homo sapiens)
Orthologous Events
ATXN3 binds RAD23 (Bos taurus)
ATXN3 binds RAD23 (Caenorhabditis elegans)
ATXN3 binds RAD23 (Canis familiaris)
ATXN3 binds RAD23 (Gallus gallus)
ATXN3 binds RAD23 (Mus musculus)
ATXN3 binds RAD23 (Plasmodium falciparum)
ATXN3 binds RAD23 (Rattus norvegicus)
ATXN3 binds RAD23 (Sus scrofa)
Authored
Jupe, S (2015-04-16)
Reviewed
Meldal, BH (2016-05-16)
Created
Jupe, S (2015-04-20)
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