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Symbol USP19 contributors: mct/pgu - updated : 28-01-2020
HGNC name ubiquitin specific protease 19
HGNC id 12617
Location 3p21.31      Physical location : 49.146.107 - 49.158.213
Synonym name
  • ubiquitin carboxyl-terminal hydrolase 19
  • zinc finger MYND domain-containing protein
  • ubiquitin thioesterase 19
  • deubiquitinating enzyme 19
  • Synonym symbol(s) KIAA0891, ZMYND9
    TYPE functioning gene
    SPECIAL FEATURE arranged in tandem
    STRUCTURE 12.89 kb     26 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Map pter - D3S3582 - USP19 - D3S1578 - D3S1588 - cen
    Authors Gene Map (98)
    Physical map
    LOC344651 3p21.31 hypothetical LOC344651 SLC26A6 3p21 solute carrier family 26, member 6 CELSR3 3p24.1-p21.2 cadherin, EGF LAG seven-pass G-type receptor 3 (flamingo homolog, Drosophila) AF3P21 3p21 cadherin, EGF LAG seven-pass G-type receptor 3 (flamingo homolog, Drosophila) IHPK2 3p21.31 inositol hexaphosphate kinase 2 PRKAR2A 3p21.3-p21.2 protein kinase, cAMP-dependent, regulatory, type II, alpha SLC25A20 3p21.31 solute carrier family 25 (carnitine/acylcarnitine translocase), member 20 ARIH2 3p21.2-p21.3 ariadne homolog 2 (Drosophila) PH-4 3p21.31 hypoxia-inducible factor prolyl 4-hydroxylase WDR6 15q21 WD repeat domain 6 FLJ10496 3p21.31 hypothetical protein FLJ10496 DKFZP564J0123 3p21.31 nuclear protein E3-3 IMPDH2 3p21.2 IMP (inosine monophosphate) dehydrogenase 2 FLJ20259 3p21.31 FLJ20259 protein QARS 3p21.3-p21.2 glutaminyl-tRNA synthetase USP19 3p21.3-p14.2 ubiquitin specific protease 19 LAMB2 3p21.3-p21.2 laminin, beta 2 (laminin S) FLJ12800 3p21.31 hypothetical protein FLJ12800 MGC35097 3p21.31 hypothetical protein MGC35097 LOC339834 3p21.31 hypothetical protein LOC339834 FLJ43654 3p21.31 FLJ43654 protein USP4 3p21.31 ubiquitin specific protease 4 (proto-oncogene) GPX1 3p21.2 glutathione peroxidase 1 ARHA 3p21.2 ras homolog gene family, member A TCTA 3p21 T-cell leukemia translocation altered gene AMT 3p21.2 aminomethyltransferase (glycine cleavage system protein T) NICN1 3p21.31 nicolin 1 DAG1 3p21.2-p21.1 dystroglycan 1 (dystrophin-associated glycoprotein 1) BSN 3p21.31 bassoon (presynaptic cytomatrix protein)
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    26 - 4401 145.6 1318 - 2019 30386869
    26 - 4492 - 1268 - 2019 30386869
    26 - 4498 - 1270 - 2019 30386869
    27 - 4795 - 1369 - 2019 30386869
    27 - 4820 - 1371 - 2019 30386869
    27 - 4847 - 1372 - 2019 30386869
    26 - 4537 - 1283 - 2019 30386869
    26 - 4537 - 1283 - 2019 30386869
    27 - 4878 - 1419 - 2019 30386869
    27 - 4883 - 1384 - 2019 30386869
    27 - 4831 - 1381 - 2019 30386869
    27 - 4867 - 1420 - 2019 30386869
    27 - 4840 - 1384 - 2019 30386869
    26 - 4543 - 1285 - 2019 30386869
    27 - 4714 - 1369 - 2019 30386869
    Type ubiquitous
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Nervousnerve   highly
    Skin/Tegumentskin   highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectivebone  highly
    Muscularstriatumskeletal highly
    cell lineage
    cell lines
    at STAGE
  • only deubiquitinating enzyme containing a C-terminal transmembrane domain, that appears to be partially stabilized in the cytosol by an interaction with its own catalytic domain, resulting in auto-inhibition of its deubiquitinating activity
  • deubiquitin family
  • peptidase C19 family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
  • like RNF123, is localized to the cytosol
  • tail-anchored ubiquitin-specific protease localized to the ER
  • basic FUNCTION
  • involved in the ubiquitin-dependent proteolytic pathway in conjunction with the 26S proteasome
  • deubiquitinating enzyme that regulates the stability of a cyclin-dependent kinase inhibitor and demonstrate that progression through G1 to S phase is, like the metaphase-anaphase transition, controlled in a hierarchical, multilayered fashion
  • rescues the ER-associated-degradation (ERAD) substrates CFTRDelta508 and TCR-alpha from proteasomal degradation
  • function on specific ERAD substrates
  • modulates transcription of major myofibrillar proteins and the ubiquitin system not only mediates the increased protein breakdown but is also involved in the decreased protein synthesis in atrophying skeletal muscle
  • positively regulates proliferation in fibroblasts by stabilizing RNF123, a ubiquitin ligase for CDKN1B
  • regulates hypoxia-inducible factor 1A (HIF1A) during hypoxia
  • only the ER-localized isoform of USP19 (USP19-ER) modulated myoblast fusion as well as the expression of myogenin and myofibrillar proteins, and these effects were also dependent on USP19 catalytic activity
  • USP19 is involved in human muscle wasting
  • possible function of USP19 in quality control of misfolded proteins by regulating their protein levels
  • USP19 is involved in the regulation of ERAD by controlling the stability of MARCHF6 via deubiquitination
  • dual functions of the USP19-BECN1 axis by balancing autophagy and the production of type IIFNs
  • USP19 is a key factor in modulating DNA damage repair by targeting HDAC1/2 K63-linked ubiquitination
  • USP19 is an important regulator of fat development
    a component
    small molecule
  • interacting with and stabilizing RNF123
  • USP19, a deubiquitinating enzyme, interacts with cellular BIRC2, BIRC3
  • interacts with components of the hypoxia pathway including HIF1A and rescues it from degradation independent of its catalytic activity
  • SIAH1 and SIAH2 are binding partners of USP19, interaction mediated by a SIAH-consensus binding motif and promoting USP19 ubiquitylation and proteasome-dependent degradation
  • regulation of CORO2A through the deubiquitinating activity of USP19 affected the transcriptional repression activity of the retinoic acid receptor (RAR), suggesting that USP19 may be involved in the regulation of RAR-mediated adipogenesis
  • USP19 is the only deubiquitinase that directly binds to SIAHs(SIAH1, SIAH2) through the substrate binding pocket
  • USP19 regulates the stability of SYVN1 and provide insight into the regulatory mechanism of the ERAD ubiquitin ligases
  • DNAJC5 facilitates USP19-dependent unconventional secretion of misfolded cytosolic proteins
  • mechanistically, USP19 interacted with MAP3K7 in a TNF or IL1B-dependent manner
  • USP19 interacts with TICAM1 and catalyzes the removal of TICAM1 K27-linked polyubiquitin moieties, thereby impairing the recruitment of TICAM1 to TLR3/4
  • cell & other
    induced by in skeletal muscle atrophying in response to numerous catabolic stimuli
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    its inactivation leads to protection from muscle loss induced by stimuli that are common in many illnesses causing cachexia
    Variant & Polymorphism
    Candidate gene
    Therapy target
    inhibition of USP19 could have therapeutic potential to protect from the deleterious consequences of obesity and diabetes
    inhibition of USP19 may be a useful approach to the treatment of many muscle-wasting conditions