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FLASH GENE
Symbol NVL contributors: mct - updated : 17-03-2020
HGNC name nuclear VCP-like
HGNC id 8070
Location 1q41      Physical location : 224.415.036 - 224.517.872
Genatlas name nuclear valosin-containing protein-like
Synonym symbol(s) NVL2
DNA
TYPE like-sequence
STRUCTURE 102.84 kb     23 Exon(s)
Genomic sequence alignment details
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked   status confirmed
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
23 splicing 2921 95.05 856 mainly localized in the nucleolus where it is involved in the biogenesis of the 60S ribosomal subunit 2012 22226966
  • NVL2
  • not phosphorylated
  • nucleoplasmic
  • two ATPase domains, each of which contains the conserved lysine residues, required for ATP-binding activity
  • in addition to its participation in ribosome biogenesis, is required for telomerase assembly and has a unique and independent role in the regulation of telomerase activity
  • full-length NVL1 and NVL2 were found to associate with TERT while other truncated forms failed to interact with TERT
  • may participate in telomerase biogenesis
  • 22 splicing 2847 82.62 750 present in the nucleoplasm 2012 22226966
  • NVL1
  • phosphorylated
  • the major species, is mainly present in the nucleolus
  • two nuclear localization signals, one of which is shared with NVL2, a nucleolar localization signal was found to exist in the N-terminal extra region
  • full-length NVL1 and NVL2 were found to associate with TERT while other truncated forms failed to interact with TERT
  • - - 2667 - 765 - 2012 22226966
    - - 2616 - 667 - 2012 22226966
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveesophagus   highly
     mouthtongue  highly
    Endocrinepancreas   lowly
    Lymphoid/Immunethymus   highly
    Nervousbrain   moderately
    Reproductivemale systemprostate  lowly
    Respiratorylung   moderately
    Urinarykidney   moderately
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectiveadipose  highly
    Epithelialsecretoryglandularendocrine 
    Epithelialsecretoryglandularexocrine 
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • AAA family chaperone-like (adenosine triphosphatases associated with diverse cellular activities)
  • a small globular domain, which contains a “classic” nucleolar localization signal consensus sequence, (R/K)(R/K)X(R/K)
  • secondary structure
  • N-terminal unique domain contains three alpha helices with an organization resembling that of a winged helix motif, whereas a pair of beta-strands is missing
  • HOMOLOGY
    Homologene
    FAMILY
  • chaperone-like AAA-ATPase family
  • CATEGORY chaperone/stress , enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,nucleus,nucleolus
    text
  • inner membrane
  • NVL interacts and co-localizes with TERT in the nucleolus
  • basic FUNCTION
  • acting as an ATP-dependent zinc metallopeptidase
  • involved in the degradation of sigma-32
  • might facilitate the dissociation and recycling of nucleolin, thereby promoting efficient ribosome biogenesis
  • physiological function for NVL in the nucleolus
  • dissociates the nucleolin-RNA complex to promote ribosome biogenesis
  • in addition to its role in ribosome biosynthesis, is essential for telomerase biogenesis and provides an alternative approach for inhibiting telomerase activity in cancer
  • NVL is involved in pre-rRNA processing by associating with the nuclear exosome complex and MPP6 is required for maintaining the integrity of this rRNA processing complex
  • spatiotemporal regulation of ribosome biogenesis in the nucleolus is mediated by the ATPase activity of NVL
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • NVL is a component of pre-ribosomal particles that interacts with the DExD/H-box RNA helicase SKIV2L2
  • INTERACTION
    DNA
    RNA
    small molecule metal binding, nucleotide,
  • 1 zinc ion
  • ATP binding
  • protein
  • interaction with RPL5, ATP-dependent and likely contributing to the nucleolar translocation of NVL
  • binding partner of NVL
  • is a novel TERT-interacting protein (NVL interacts and co-localizes with TERT in the nucleolus)
  • NVL is a remodeling factor for the MTREX-exosome complex during the maturation of pre-ribosomal particles
  • WDR74 is a factor that specifically dissociates from the complex depending on the ATPase activity of NVL
  • WDR74 is a novel regulatory protein of the MTREX-exsosome complex whose interaction is regulated by NVL and is involved in ribosome biogenesis
  • WDR74 associates with the D1 AAA domain of NVL, which represents a novel mode of binding of a substrate with a type II AAA-ATPase
  • 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
  • WDR74 is a MTREX-interacting partner, whose dissociation is stimulated by the ATP hydrolysis of NVL
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancer  
    NVL2 represents a new pathway for regulating the telomerase assembly as well as its enzymatic activity and provides an alternative approach for inhibiting telomerase activity in cancer
    ANIMAL & CELL MODELS