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Gene Review

USP11  -  ubiquitin specific peptidase 11

Homo sapiens

Synonyms: Deubiquitinating enzyme 11, UHX1, Ubiquitin carboxyl-terminal hydrolase 11, Ubiquitin thioesterase 11, Ubiquitin-specific-processing protease 11
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Disease relevance of USP11


Psychiatry related information on USP11


High impact information on USP11

  • BRCA2 is ubiquitinated in vivo and interacts with USP11, a deubiquitinating enzyme that exhibits prosurvival function in the cellular response to DNA damage [2].
  • Sequencing revealed that USP11 possesses Cys box, His box, Asp and KRF domains, which are highly conserved in many ubiquitin-specific proteases [3].
  • The full-length cDNA of USP11 was cloned from a Jurkat cell library [3].
  • By immunoblotting using HeLa cells, we concluded that 921-residue version of USP11 was the predominant form, and USP11 may be a ubiquitous protein in various human tissues [3].
  • Thus mutations of UHX1, and probably PCTK1, do not appear to cause common X-linked eye diseases [1].

Biological context of USP11

  • Antagonism of USP11 function either through expression of this mutant or through RNA interference increased cellular sensitivity to MMC in a BRCA2-dependent manner [2].
  • Reevaluation of the UHX1 gene structure demonstrated five new exons, for a total of 21 exons and a predicted protein product of 963 amino acids [1].
  • Evaluation of patients revealed no UHX1 mutations using SSCP (10 CSNB1 and 20 XLRP) or deletion screening with cDNA hybridisation (13 CSNB1 and 43 XLRP) [1].

Other interactions of USP11

  • The ectopic expression of IKKalpha into cells silenced for USP11 restores p53 expression, demonstrating that USP11 functions as an upstream regulator of an IKKalpha-p53 signaling pathway [4].

Analytical, diagnostic and therapeutic context of USP11

  • By immunofluorescence assay, USP11 primarily was localized in the nucleus of non-dividing cells, suggesting an association between USP11 and RanBPM in the nucleus [3].


  1. UHX1 and PCTK1: precise characterisation and localisation within a gene-rich region in Xp11.23 and evaluation as candidate genes for retinal diseases mapped to Xp21.1-p11.2. Brandau, O., Nyakatura, G., Jedele, K.B., Platzer, M., Achatz, H., Ross, M., Murken, J., Rosenthal, A., Meindl, A. Eur. J. Hum. Genet. (1998) [Pubmed]
  2. BRCA2 is ubiquitinated in vivo and interacts with USP11, a deubiquitinating enzyme that exhibits prosurvival function in the cellular response to DNA damage. Schoenfeld, A.R., Apgar, S., Dolios, G., Wang, R., Aaronson, S.A. Mol. Cell. Biol. (2004) [Pubmed]
  3. Structural and functional characterization of the USP11 deubiquitinating enzyme, which interacts with the RanGTP-associated protein RanBPM. Ideguchi, H., Ueda, A., Tanaka, M., Yang, J., Tsuji, T., Ohno, S., Hagiwara, E., Aoki, A., Ishigatsubo, Y. Biochem. J. (2002) [Pubmed]
  4. The deubiquitinating enzyme USP11 controls an IkappaB kinase alpha (IKKalpha)-p53 signaling pathway in response to tumor necrosis factor alpha (TNFalpha). Yamaguchi, T., Kimura, J., Miki, Y., Yoshida, K. J. Biol. Chem. (2007) [Pubmed]
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