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Symbol USP7 contributors: mct/shn - updated : 06-11-2016
HGNC name ubiquitin specific protease 7 (herpes virus-associated)
HGNC id 12630
Location 16p13.2      Physical location : 8.985.950 - 9.057.341
Synonym name
  • Herpes virus-associated ubiquitin-specific protease
  • deubiquitinating enzyme 7
  • herpesvirus-associated ubiquitin-specific protease
  • ubiquitin carboxyl-terminal hydrolase 7
  • ubiquitin specific protease 7 (herpes virus-associated)
  • ubiquitin thioesterase 7
  • ubiquitin-specific-processing protease 7
  • Synonym symbol(s) HAUSP, TEF1
    TYPE functioning gene
    STRUCTURE 71.39 kb     31 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked   status provisional
    Map pter - D16S406 - D16S3020 - USP7 - D16S3087 - D16S404 - cen
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    31 - 5412 - 1102 - 2012 22361354
    - - - - - - 2012 22361354
  • phosphorylation stabilizes USP7S and thus contributes to MDM2 stabilization and downregulation of TP53
  • dephosphorylation of USP7S by the ATM-dependent protein phosphatase PPM1G leads to USP7S downregulation, followed by MDM2 downregulation and accumulation of TP53
    Type ubiquitous
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Lymphoid/Immunelymph node   highly
    Reproductivemale systemtestis  highly
    Respiratoryrespiratory tractlarynx  highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    fluid/secretion lymph
    at STAGE
  • a MATH domain
  • a p53 binding domain
  • a catalytic domain,
  • two C-terminal domains
  • an N-terminal TRAF-like domain
  • mono polymer dimer
    interspecies ortholog to Usp7, Rattus norvegicus
    ortholog to USP7, Pan troglodytes
    ortholog to usp7, Danio rerio
    ortholog to Usp7, Mus musculus
  • deubiquitin peptidase family C19
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus,nucleoplasm,nuclear bodies
    text interacting with MARCH7 in the nucleus
    basic FUNCTION
  • ubiquitin-specific protease, stimulating viral gene expression
  • playing a role in the pathogenesis of spinocerebellar ataxia type 1
  • deubiquitylating enzyme playing a role not only in transcriptional regulation but also in DNA replication, apoptosis, and possibly endosomal organization
  • has an important role in regulating FOXO-mediated stress responses
  • can stabilize MARCH7, and deubiquitylate MARCH7 in the nucleus
  • deubiquitinase that critically regulates the TP53-MDM2 pathway and modulates the TP53 function, inducing TP53-dependent cell growth repression and apoptosis
  • USP7 is a deubiquitinating enzyme that regulates the ubiquitination state of RNF2
  • stabilizes REST through deubiquitylation and antagonizes beta-TrCP in regulating REST at the post-translational level
  • counterbalances REST ubiquitylation and prevents neural stem/progenitor cells (NPCs) differentiation, and USP7-mediated deubiquitylation represents a critical regulatory mechanism involved in the maintenance of NPCs
  • involved in the cellular stress response by regulating MDM2 and TP53 protein levels following severe DNA damage
  • stimulates repair of oxidative DNA lesions
  • has a critical role in regulating the TP53-MDM2 (murine double minute 2) pathway
  • USP7-mediated TP53 regulation is crucial for brain development
  • dual role of USP7, regulating the protein turnover of UHRF1 and stimulating the enzymatic activity of DNMT1
  • regulates the stability of TP53 and the TP53-binding protein MDM2
  • plays a major role in regulating genome stability and cancer prevention by controlling the key proteins involved in the DNA damage response
  • its regulation plays a central role in DNA damage signal transmission
  • UVSSA, together with USP7, is implicated in regulating
  • Transcription-coupled nucleotide excision repair (TC-NER) activity
  • NMRAL1 and USP7 function in concert in inhibiting polyubiquination of IKBKG, thus inhibiting NFKB activity
  • is a protein deubiquitinase with an essential role in development
  • USP7 may act as an oncogenic protein that can modulate MYC expression via TRRAP
  • is a novel component of the cellular DDR involved in preserving the genome stability
  • TRIM32 associates with the deubiquitination enzyme USP7, which previously has been implicated in neural stem cell maintenance, and exhibit a dynamic and partially overlapping expression pattern during neuronal differentiation
  • SCML2/USP7 complex constitutes a novel molecular pathway in modulating the epigenetic state of sex chromosomes during male meiosis
  • a component
  • transitory constituent of discrete nuclear structures (ND10)
  • part of USP7-HLTF-PCNA molecular network controlling DNA damage response
  • complexing with TRIM27 and ubiquitination-deubiquitination cascade mediated by the TRIM27-USP7 complex plays an important role in TNF-induced apoptosis
    small molecule
  • polyubiquitinated, polyneddylated, and dimerized
  • protein
  • binding herpes virus type 1 immediate early protein Vmw 110
  • p53
  • ataxin 1, SCA1
  • guanine monphosphate synthetase, GMPS
  • EBNA1 peptide
  • a key regulator of p53 and Mdm2
  • Mdm4 p53 binding protein homolog (mouse), MDM4
  • forkhead box O4, FOXO4
  • checkpoint with forkhead and ring finger domains, CHFR
  • Claspin, CLSPN
  • Polycomb repressive complex 1, PRC1; melanoma nuclear protein 18, MEL18; BMI1 polycomb ring finger oncogene, BMI1
  • TSPYL5 interacts with USP7(TSPYL5 reduces the activity of USP7 towards TP53, resulting in increased TP53 ubiquitylation)
  • both DNMT1 and UHRF1 coprecipitate with ubiquitin specific peptidase 7 (USP7)
  • ING1 physically interacts with herpesvirus-associated ubiquitin-specific protease (USP7)
  • interaction partner of DNMT1 and UHRF1
  • is a novel regulator of HLTF stability
  • USP7 interacts with UHRF1 (both physical interaction and catalytic activity of USP7 are necessary for UHRF1 ubiquitylation and stability regulation)
  • UVSSA-USP7–mediated stabilization of ERCC6 represents a critical regulatory mechanism of TC-NER in restoring gene expression
  • UVSSA forms a complex with USP7, stabilizes ERCC6 and restores the hypophosphorylated form of RNA polymerase II after UV irradiation
  • USP7 is a novel interacting protein of the oligonucleotide/oligosaccharide-binding fold of TPP1, which was previously known to recruit telomerase to telomeres
  • USP7 cooperates with SCML2 to regulate the activity of PRC1
  • TRRAP physically interact and co-localize with USP7
  • de-ubiquitylating enzyme USP7 is a critical regulator of RAD18 protein levels
  • KMT2E protein stability is cooperatively regulated by O-GlcNAc transferase (OGT) and ubiquitin-specific protease 7 (USP7)
  • ATF4 could maintain SREBF1 protein stability by directly activating the expression of USP7 which deubiquitinates SREBF1 and increases its protein content in cell
  • balanced ubiquitination and deubiquitination of MYC by TRIM32 and USP7 is a novel mechanism for stem cell fate determination
  • cell & other
    inhibited by bilateral inhibition of USP7 deubiquitinase by a viral interferon regulatory factor protein
    corresponding disease(s)
    Variant & Polymorphism
    Candidate gene
    Therapy target
    therapeutic target for tuning TP53-mediated antitumor activity
  • neural cell survival and brain development of hausp-mutant mice can largely be restored in the Tp53-null background