Homo sapiens         DUB/Josephin


※ DUB/Josephin family introduction

    Josephin family represents DUBs with a papain-like cysteine protease catalytic domain. As a well studied Josephin family protein of Ataxin-3, the structure of the Josephin domain was solved by NMR which resembles that of UCH domain deubiquitinating enzymes. Furthermore, the active site residues of Ataxin-3 are positioned in a catalytically competent conformation which has been reported to edit K63-linked chains specifically (1).

1. Reyes-Turcu, F.E., Ventii, K.H. and Wilkinson, K.D. (2009) Regulation and cellular roles of ubiquitin-specific deubiquitinating enzymes. Annu. Rev. Biochem., 78, 363-397. PMID: 19489724




There are 4 genes.  Reviewed (4 or Unreviewed (0Download the gene list
StatusiUUCD IDEnsemble Gene IDUniProt AccessionGene Name
IUUC-Hsa-046522
ENSG00000161677.11
Q8TAC2
JOSD2; SBBI54
IUUC-Hsa-046591
ENSG00000066427.21
P54252
ATXN3; ATX3; MJD; MJD1; SCA3
IUUC-Hsa-047013
ENSG00000123594.5
Q9H3M9
ATXN3L; ATX3L; MJDL
IUUC-Hsa-047008
ENSG00000100221.10
Q15040
JOSD1; JSPH1; KIAA0063