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Title: Usp6  
Author: World Heritage Encyclopedia
Language: English
Subject: DNAJC13, DNAJB4, DNAJC10, DNAJC11, Ubiquitin
Publisher: World Heritage Encyclopedia


Ubiquitin specific peptidase 6
Symbols  ; HRP1; TRE17; TRE2; TRESMCR; Tre-2; USP6-short
External IDs GeneCards:
EC number
RNA expression pattern
Species Human Mouse
Entrez n/a
Ensembl n/a
UniProt n/a
RefSeq (mRNA) n/a
RefSeq (protein) n/a
Location (UCSC) n/a
PubMed search n/a

Ubiquitin carboxyl-terminal hydrolase 6 is an enzyme that in humans is encoded by the USP6 gene.[1][2][3]

Aneurysmal bone cyst can be associated with a TRE17/USP6 translocation.[4]


  1. ^ Puente XS, Sanchez LM, Overall CM, Lopez-Otin C (Jul 2003). "Human and mouse proteases: a comparative genomic approach". Nat Rev Genet 4 (7): 544–58.  
  2. ^ Hoogendijk JE, Hensels GW, Gabreels-Festen AA, Gabreels FJ, Janssen EA, de Jonghe P, Martin JJ, van Broeckhoven C, Valentijn LJ, Baas F, et al. (May 1992). "De-novo mutation in hereditary motor and sensory neuropathy type I". Lancet 339 (8801): 1081–2.  
  3. ^ "Entrez Gene: USP6 ubiquitin specific peptidase 6 (Tre-2 oncogene)". 
  4. ^ Ye Y, Pringle LM, Lau AW, et al. (June 2010). "TRE17/USP6 oncogene translocated in aneurysmal bone cyst induces matrix metalloproteinase production via activation of NFκB". Oncogene 29 (25): 3619–29.  

Further reading

  • D'Andrea A, Pellman D (1999). "Deubiquitinating enzymes: a new class of biological regulators". Crit. Rev. Biochem. Mol. Biol. 33 (5): 337–52.  
  • Nakamura T, Hillova J, Mariage-Samson R, et al. (1992). "A novel transcriptional unit of the tre oncogene widely expressed in human cancer cells". Oncogene 7 (4): 733–41.  
  • Papa FR, Hochstrasser M (1993). "The yeast DOA4 gene encodes a deubiquitinating enzyme related to a product of the human tre-2 oncogene". Nature 366 (6453): 313–9.  
  • Onno M, Nakamura T, Hillova J, Hill M (1993). "Identification of novel sequences in the repertoire of hypervariable TRE17 genes from immortalized nonmalignant and malignant human keratinocytes". Gene 131 (2): 209–15.  
  • Onno M, Nakamura T, Mariage-Samson R, et al. (1993). "Human TRE17 oncogene is generated from a family of homologous polymorphic sequences by single-base changes". DNA Cell Biol. 12 (2): 107–18.  
  • Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903.  
  • Paulding CA, Ruvolo M, Haber DA (2003). "The Tre2 (USP6) oncogene is a hominoid-specific gene". Proc. Natl. Acad. Sci. U.S.A. 100 (5): 2507–11.  
  • Masuda-Robens JM, Kutney SN, Qi H, Chou MM (2003). "The TRE17 Oncogene Encodes a Component of a Novel Effector Pathway for Rho GTPases Cdc42 and Rac1 and Stimulates Actin Remodeling". Mol. Cell. Biol. 23 (6): 2151–61.  
  • Bizimungu C, De Neve N, Burny A, et al. (2003). "Expression in a RabGAP yeast mutant of two human homologues, one of which is an oncogene". Biochem. Biophys. Res. Commun. 310 (2): 498–504.  
  • Oliveira AM, Hsi BL, Weremowicz S, et al. (2004). "USP6 (Tre2) fusion oncogenes in aneurysmal bone cyst". Cancer Res. 64 (6): 1920–3.  
  • Oliveira AM, Perez-Atayde AR, Inwards CY, et al. (2004). "USP6 and CDH11 oncogenes identify the neoplastic cell in primary aneurysmal bone cysts and are absent in so-called secondary aneurysmal bone cysts". Am. J. Pathol. 165 (5): 1773–80.  
  • Martinu L, Masuda-Robens JM, Robertson SE, et al. (2004). "The TBC (Tre-2/Bub2/Cdc16) Domain Protein TRE17 Regulates Plasma Membrane-Endosomal Trafficking through Activation of Arf6". Mol. Cell. Biol. 24 (22): 9752–62.  
  • Oliveira AM, Perez-Atayde AR, Dal Cin P, et al. (2005). "Aneurysmal bone cyst variant translocations upregulate USP6 transcription by promoter swapping with the ZNF9, COL1A1, TRAP150, and OMD genes". Oncogene 24 (21): 3419–26.  
  • Bizimungu C, Vandenbol M (2005). "At least two regions of the oncoprotein Tre2 are involved in its lack of GAP activity". Biochem. Biophys. Res. Commun. 335 (3): 883–90.  
  • Shen C, Ye Y, Robertson SE, et al. (2006). "Calcium/calmodulin regulates ubiquitination of the ubiquitin-specific protease TRE17/USP6". J. Biol. Chem. 280 (43): 35967–73.  
  • Dechamps C, Bach S, Portetelle D, Vandenbol M (2006). "The Tre2 oncoprotein, implicated in Ewing's sarcoma, interacts with two components of the cytoskeleton". Biotechnol. Lett. 28 (4): 223–31.  

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