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FLASH GENE
Symbol MTREX contributors: mct - updated : 09-11-2021
HGNC name Mtr4 exosome RNA helicase
HGNC id 18734
Location 5q11.2      Physical location : -
Synonym name
  • KIAA0052 protein
  • ATP-dependent helicase SKIV2L2
  • functional spliceosome-associated protein 118
  • superkiller viralicidic activity 2-like 2 (S. cerevisiae)
  • Synonym symbol(s) Dob1, Mtr4, KIAA0052, fSAP118, SKIV2L2, MGC142069
    EC.number 3.6.4.13
    DNA
    TYPE functioning gene
    STRUCTURE 117.83 kb     27 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    27 - 4222 - 1042 - 2021 33857361
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinesmall intestine  lowly
    Lymphoid/Immunethymus     Homo sapiens
    Reproductivefemale systembreastmammary gland moderately
     male systemtestis  moderately
     male systemtestis    Mus musculusAdult
    Respiratoryrespiratory tracttrachea  highly
    Urinarybladder   moderately
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoieticbone marrow  moderately
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Reproductivespermatid Mus musculusAdult
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period embryo, pregnancy
    Text embryonic tissue
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • DEAD box, conserved motif Asp-Glu-Ala-Asp (DEAD)
  • an helicase ATP-binding domain
  • an helicase C-terminal domain
  • HOMOLOGY
    interspecies homolog to murine Skiv2l2 (97.50 pc)
    homolog to fission yeast Nrl2
    intraspecies homolog to SKI2
    Homologene
    FAMILY
  • helicase family
  • Ski2 family within the superfamily 2 of RNA helicases
  • CATEGORY immunity/defense
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus,nucleolus
    basic FUNCTION
  • involved in antiviral activity by blocking translation of poly(A) deficient mRNAs
  • may be involved in pre-mRNA splicing
  • INTS1, TRRAP, and SKIV2L2 are implicated in distinct steps of thymus development
  • has both the RNA-binding and ATPase activities
  • plays a key role in specifically recruiting the exosome to its targets
  • NRDE2 and MTREX play a role in the DNA damage response by maintaining low DNA double-strand break levels
  • is a RNA helicase associated with a nuclear exosome that plays key roles in RNA processing and surveillance
  • is a mediator of the functions of MYC in cancer metabolism
  • is a SKI2-like RNA helicase that plays a central role in RNA surveillance and degradation pathways as an activator of the RNA exosome
  • is at the center of a dynamic process that recruits RNA substrates and presents them to the exosome
  • CELLULAR PROCESS nucleotide, RNA splicing
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component part of the trimeric Nuclear Exosome Targeting (NEXT) complex, containing SKIV2L2, the Zn-knuckle protein ZCCHC8, and the putative RNA binding protein RBM7
    INTERACTION
    DNA binding
    RNA binding
    small molecule nucleotide,
  • ATP binding
  • protein
  • NVL is a remodeling factor for the MTREX-exosome complex during the maturation of pre-ribosomal particles
  • WDR74 is a novel regulatory protein of the MTREX-exsosome complex whose interaction is regulated by NVL and is involved in ribosome biogenesis
  • competition between MTREX and ALYREF determines exosome recruitment and functions in creating balanced nuclear RNA pools for degradation and export
  • WDR74 associates with MTREX, the nuclear exosome-assisting RNA helicase, whose dissociation is prohibited by an ATPase-deficient mutant of the AAA-type chaperone NVL
  • dissociation of WDR74 from MTREX in a late stage of rRNA synthesis is thought to be required for appropriate maturation of the pre-60S particles
  • MTREX association with NRDE2, defines a novel function for MTREX in the DNA damage response pathway
  • NRDE2 inhibits MTREX recruitment and RNA degradation, and thereby ensures efficient mRNA nuclear export
  • WDR74 is a MTREX-interacting partner, whose dissociation is stimulated by the ATP hydrolysis of NVL
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in hepatocellular carcinoma (HCC)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
  • independent diagnostic marker predicting the poor prognosis of HCC patients
  • Therapy target
    SystemTypeDisorderPubmed
    cancerdigestiveliver
    as a promising therapeutic target for treating HCC
    ANIMAL & CELL MODELS