Gene Symbol: Usp9x
Description: ubiquitin specific peptidase 9, X chromosome
Alias: 5730589N07Rik, AA407302, AA407699, AL022658, AL022749, Dffrx, FAF-X, Fafl, deubiquitinating enzyme FAF-X, fat facets homolog, fat facets protein-related, X-linked, fats facets protein related, X, ubiquitin carboxyl-terminal hydrolase FAM, ubiquitin specific protease 9 SSSRF- isoform, ubiquitin thioesterase FAF-X, ubiquitin thiolesterase FAF-X, ubiquitin-specific protease 9, X chromosome, ubiquitin-specific-processing protease FAF-X
Species: mouse
Products:     Usp9x

Top Publications

  1. Sato T, Kanai Y, Noma T, Kanai Azuma M, Taya S, Matsui T, et al. A close correlation in the expression patterns of Af-6 and Usp9x in Sertoli and granulosa cells of mouse testis and ovary. Reproduction. 2004;128:583-94 pubmed
    b>Usp9x, an X-linked deubiquitylating enzyme, is stage dependently expressed in the supporting cells (i.e. Sertoli cells and granulosa cells) and germ cells during mouse gametogenesis...
  2. Wood S, Pascoe W, Ru K, Yamada T, Hirchenhain J, Kemler R, et al. Cloning and expression analysis of a novel mouse gene with sequence similarity to the Drosophila fat facets gene. Mech Dev. 1997;63:29-38 pubmed
    ..The strong sequence similarity to faf and the developmentally regulated expression pattern suggest that Fam and the ubiquitin pathway may play a role in determining cell fate in mammals, as has been established for Drosophila. ..
  3. Homan C, Kumar R, Nguyen L, Haan E, Raymond F, Abidi F, et al. Mutations in USP9X are associated with X-linked intellectual disability and disrupt neuronal cell migration and growth. Am J Hum Genet. 2014;94:470-8 pubmed publisher
    ..X-linked intellectual disability, we identified three unique variants (two missense and one protein truncating) in USP9X. To assess the functional significance of these variants, we took advantage of the Usp9x knockout mouse we ..
  4. Stegeman S, Jolly L, Premarathne S, Gecz J, Richards L, Mackay Sim A, et al. Loss of Usp9x disrupts cortical architecture, hippocampal development and TGF?-mediated axonogenesis. PLoS ONE. 2013;8:e68287 pubmed publisher
    The deubiquitylating enzyme Usp9x is highly expressed in the developing mouse brain, and increased Usp9x expression enhances the self-renewal of neural progenitors in vitro...
  5. Park Y, Jin H, Liu Y. Regulation of T cell function by the ubiquitin-specific protease USP9X via modulating the Carma1-Bcl10-Malt1 complex. Proc Natl Acad Sci U S A. 2013;110:9433-8 pubmed publisher
    ..Here we show that in vivo knockdown of the deubiquitinating enzyme USP9X attenuates T-cell proliferation...
  6. Perez Mancera P, Rust A, van der Weyden L, Kristiansen G, Li A, Sarver A, et al. The deubiquitinase USP9X suppresses pancreatic ductal adenocarcinoma. Nature. 2012;486:266-70 pubmed publisher
    ..The most commonly mutated gene was the X-linked deubiquitinase Usp9x, which was inactivated in over 50% of the tumours...
  7. Th ard D, Labarrade F, Partisani M, Milanini J, Sakagami H, Fon E, et al. USP9x-mediated deubiquitination of EFA6 regulates de novo tight junction assembly. EMBO J. 2010;29:1499-509 pubmed publisher
    ..We report here that the deubiquitinating enzyme USP9x has a critical function in TJ biogenesis by controlling the levels of the exchange factor for Arf6 (EFA6), a ..
  8. Dupont S, Mamidi A, Cordenonsi M, Montagner M, Zacchigna L, Adorno M, et al. FAM/USP9x, a deubiquitinating enzyme essential for TGFbeta signaling, controls Smad4 monoubiquitination. Cell. 2009;136:123-35 pubmed publisher
    ..By means of siRNA screen we identified FAM (USP9x), a deubiquitinase acting as essential and evolutionarily conserved component in TGFbeta and bone morphogenetic ..
  9. Friocourt G, Kappeler C, Saillour Y, Fauchereau F, Rodriguez M, Bahi N, et al. Doublecortin interacts with the ubiquitin protease DFFRX, which associates with microtubules in neuronal processes. Mol Cell Neurosci. 2005;28:153-64 pubmed
    ..we show that DCX interacts with the ubiquitin-specific protease Drosophila fat facets related on X chromosome (DFFRX). This interaction was confirmed by targeted mutagenesis, colocalization, and immunoprecipitation studies...

More Information


  1. Brown G, Furlong R, Sargent C, Erickson R, Longepied G, Mitchell M, et al. Characterisation of the coding sequence and fine mapping of the human DFFRY gene and comparative expression analysis and mapping to the Sxrb interval of the mouse Y chromosome of the Dffry gene. Hum Mol Genet. 1998;7:97-107 pubmed
    DFFRY (the Y-linked homologue of the DFFRX Drosophila fat-facets related X gene) maps to proximal Yq11.2 within the interval defining the AZFa spermatogenic phenotype...
  2. Jolly L, Taylor V, Wood S. USP9X enhances the polarity and self-renewal of embryonic stem cell-derived neural progenitors. Mol Biol Cell. 2009;20:2015-29 pubmed publisher
    The substrate-specific deubiquitylating enzyme USP9X is a putative "stemness" gene expressed in many progenitor cell populations...
  3. Xu J, Taya S, Kaibuchi K, Arnold A. Sexually dimorphic expression of Usp9x is related to sex chromosome complement in adult mouse brain. Eur J Neurosci. 2005;21:3017-22 pubmed
    We found the expression of Usp9x, an X-linked gene which encodes a ubiquitin protease implicated in synaptic development, to be significantly higher in the adult female mouse brains than in male brains...
  4. Hall N, Brown G, Furlong R, Sargent C, Mitchell M, Rocha D, et al. Usp9y (ubiquitin-specific protease 9 gene on the Y) is associated with a functional promoter and encodes an intact open reading frame homologous to Usp9x that is under selective constraint. Mamm Genome. 2003;14:437-47 pubmed
    Sequences complementary to the X-linked ubiquitin-specific protease gene Usp9x (Dffrx) have been shown to map to the Sxr(b) interval of the mouse Y Chromosome (chr) and to be expressed in a testis-specific manner...
  5. Jahan A, Lestra M, Swee L, Fan Y, Lamers M, Tafesse F, et al. Usp12 stabilizes the T-cell receptor complex at the cell surface during signaling. Proc Natl Acad Sci U S A. 2016;113:E705-14 pubmed publisher
    ..Our approach benefits from the use of activity-based probes in primary cells without any previous genome modification, and underscores the importance of ubiquitin-mediated regulation to refine signaling cascades. ..
  6. Naik E, Webster J, DeVoss J, Liu J, Suriben R, Dixit V. Regulation of proximal T cell receptor signaling and tolerance induction by deubiquitinase Usp9X. J Exp Med. 2014;211:1947-55 pubmed publisher
    ..Here, we define a cell intrinsic role for the deubiquitinase Usp9X during proximal TCR signaling...
  7. Kanai Azuma M, Mattick J, Kaibuchi K, Wood S. Co-localization of FAM and AF-6, the mammalian homologues of Drosophila faf and canoe, in mouse eye development. Mech Dev. 2000;91:383-6 pubmed
    ..Expression is not limited to the epithelia however, as FAM and AF-6 also co-localize during lens fibre development as well as in sub-populations of the neural retina. ..
  8. Garreau A, Blaize G, Argenty J, Rouquié N, Tourdès A, Wood S, et al. Grb2-Mediated Recruitment of USP9X to LAT Enhances Themis Stability following Thymic Selection. J Immunol. 2017;199:2758-2766 pubmed publisher
    ..We found that Themis stability is controlled by the ubiquitin-specific protease USP9X, which removes ubiquitin K48-linked chains on Themis following TCR engagement...
  9. Kishi K, Uchida A, Takase H, Suzuki H, Kurohmaru M, Tsunekawa N, et al. Spermatogonial deubiquitinase USP9X is essential for proper spermatogenesis in mice. Reproduction. 2017;154:135-143 pubmed publisher
    b>USP9X (ubiquitin-specific peptidase 9, X chromosome) is the mammalian orthologue of Drosophila deubiquitinase fat facets that was previously shown to regulate the maintenance of the germ cell lineage partially through stabilizing ..
  10. Oishi S, Premarathne S, Harvey T, Iyer S, Dixon C, Alexander S, et al. Usp9x-deficiency disrupts the morphological development of the postnatal hippocampal dentate gyrus. Sci Rep. 2016;6:25783 pubmed publisher
    ..Our understanding of the repertoire of these factors, however, remains limited. The deubiquitylating enzyme USP9X has recently emerged as a mediator of neural stem cell identity...
  11. Naik E, Dixit V. Usp9X Is Required for Lymphocyte Activation and Homeostasis through Its Control of ZAP70 Ubiquitination and PKCβ Kinase Activity. J Immunol. 2016;196:3438-51 pubmed publisher
    ..In this article, we show that Usp9X functions as a positive regulatory switch during T lymphocyte priming through removal of inhibitory ..
  12. Paemka L, Mahajan V, Ehaideb S, Skeie J, Tan M, Wu S, et al. Seizures are regulated by ubiquitin-specific peptidase 9 X-linked (USP9X), a de-ubiquitinase. PLoS Genet. 2015;11:e1005022 pubmed publisher
    ..was defined, identifying prickle-interacting proteins that localize to synapses and a novel interacting partner, USP9X, a substrate-specific de-ubiquitinase...
  13. Crozet F, El Amraoui A, Blanchard S, Lenoir M, Ripoll C, Vago P, et al. Cloning of the genes encoding two murine and human cochlear unconventional type I myosins. Genomics. 1997;40:332-41 pubmed
    ..The human orthologuous genes MYO1C and MYO1F were also characterized, and mapped to the human chromosomal regions 17p18 and 19p13.2-19p13.3, respectively. ..
  14. Huntwork Rodriguez S, Wang B, Watkins T, Ghosh A, Pozniak C, Bustos D, et al. JNK-mediated phosphorylation of DLK suppresses its ubiquitination to promote neuronal apoptosis. J Cell Biol. 2013;202:747-63 pubmed publisher
    ..of DLK ubiquitination, which was mediated by the E3 ubiquitin ligase PHR1 and the de-ubiquitinating enzyme USP9X. Abundance of DLK in turn controlled the levels of downstream JNK signaling and apoptosis...
  15. Sandstedt S, Tucker P. Evolutionary strata on the mouse X chromosome correspond to strata on the human X chromosome. Genome Res. 2004;14:267-72 pubmed
    ..An analysis of smaller fragments of Smcy, Smcx, Zfy, and Zfx from seven species of Mus confirmed that the strata in Mus musculus were representative of the genus Mus. ..
  16. Xu J, Burgoyne P, Arnold A. Sex differences in sex chromosome gene expression in mouse brain. Hum Mol Genet. 2002;11:1409-19 pubmed
    ..Six X-linked homologues (Usp9x, Ube1x, Smcx, Eif2s3x, Utx and Dbx) were also expressed in brain, and in adulthood all of these transcripts were ..
  17. Grasso D, Ropolo A, Lo Re A, Boggio V, Molejón M, Iovanna J, et al. Zymophagy, a novel selective autophagy pathway mediated by VMP1-USP9x-p62, prevents pancreatic cell death. J Biol Chem. 2011;286:8308-24 pubmed publisher
    ..The autophagy-related protein VMP1, the ubiquitin-protease USP9x, and the ubiquitin-binding protein p62 mediate zymophagy...
  18. Noma T, Kanai Y, Kanai Azuma M, Ishii M, Fujisawa M, Kurohmaru M, et al. Stage- and sex-dependent expressions of Usp9x, an X-linked mouse ortholog of Drosophila Fat facets, during gonadal development and oogenesis in mice. Gene Expr Patterns. 2002;2:87-91 pubmed
    ..Here we show that Usp9x, an X-linked ortholog of Faf, is predominantly expressed in both germ cell and supporting cell lineages during ..
  19. Xu J. Age-related changes in Usp9x protein expression and DNA methylation in mouse brain. Brain Res Mol Brain Res. 2005;140:17-24 pubmed
    b>Usp9x, a ubiquitin-specific protease implicated in synaptic development, was found to be more abundant in adult as compared to newborn mouse brain tissue...
  20. Agrawal P, Chen Y, Schilling B, Gibson B, Hughes R. Ubiquitin-specific peptidase 9, X-linked (USP9X) modulates activity of mammalian target of rapamycin (mTOR). J Biol Chem. 2012;287:21164-75 pubmed publisher
    ..In this study we identify a novel mTOR interacting protein, the ubiquitin-specific protease USP9X, which acts as a negative regulator of mTOR activity and muscle differentiation...
  21. Noma T, Kanai Y, Kanai Azuma M, Ishii M, Fujisawa M, Kurohmaru M, et al. Stage- and sex-dependent expressions of Usp9x, an X-linked mouse ortholog of Drosophila Fat facets, during gonadal development and oogenesis in mice. Mech Dev. 2002;119 Suppl 1:S91-5 pubmed
    ..Here we show that Usp9x, an X-linked ortholog of Faf, is predominantly expressed in both germ cell and supporting cell lineages during ..