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FLASH GENE
Symbol LNX1 contributors: mct - updated : 14-03-2012
HGNC name ligand of numb-protein X 1
HGNC id 6657
Location 4q12      Physical location : 54.326.438 - 54.457.724
Synonym name
  • multi-PDZ-domain-containing protein
  • ligand of numb-protein X
  • PDZ domain-containing ring finger protein 2
    • Synonym symbol(s) MPDZ, PDZRN2, LNX, PDZRN2
    EC.number 6.3.2.19
    DNA
    TYPE functioning gene
    STRUCTURE 131.29 kb     10 Exon(s)
    regulatory sequence Binding site
    MAPPING cloned Y linked N status provisional
    regionally located located between D4S1577 and D4S1594
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    11 - 3170 80 728 - -
    10 - 2930 - 632 - -
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestine    
     mouth   highly
    Endocrineparathyroid   highly
    Hearing/Equilibriumearinnercochlea highly
    Reproductivefemale systemplacenta   
     male systemprostate   
    Urinarykidneynephronrenal capsuleglomerulus 
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumskeletal  
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Digestiveepithelial cell
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • four PDZ domains which are proved to play a central role in organizing diverse cell signaling assemblies
    • and a NPXY motif
  • a ring finger
  • multiple protein binding domains involved in signal transduction
    • HOMOLOGY
    interspecies homolog to murine Lnx1
    homolog to Drosophila CG11951
    homolog to C.elegans Y67D8C.9
    Homologene
    FAMILY RING finger-type E3 ubiquitin ligase family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    text in perisynaptic Schwann cells but not in Schwann cells along the motor axon
    basic FUNCTION
  • mediates ubiquitination and subsequent proteasomal degradation of isoform p66 and isoform p72 of NUMB
  • may be important for the organization of signaling complexes in Numb-involved pathways or pathways regulated by other PTB-containing proteins
  • functioning as an E2 dependant, E3 ubiquitin ligase
  • enhancing Notch signaling
  • E3 ubiquitin ligase that can target interacting proteins for degradation through ubiquitination
  • may play a role in regulating neuregulin-1/ErbB signaling in perisynaptic Schwann cells
  • playing an important role in tumorogenesis
  • facilitates endocytosis of IGSF5 and is involved in transforming growth factor beta -induced redistribution of IGSF5 in mammary epithelial cells
  • role in neurogenesis suggesting that it may contribute to development of glial tumors
  • involved in the ubiquitylation, endocytosis and lysosomal degradation of claudins, and the turnover of TJs is regulated by ubiquitylation
  • endocytic scaffold for IGSF5 that is implicated in the reorganization of cell junctions
  • could participate in signal transduction, such as Notch pathway, and play an important role in tumorigenesis
  • LNX1 or LNX2 promoted CD8A ubiquitylation, downregulation from the plasma membrane, transport to the lysosomes, and degradation
    • CELLULAR PROCESS protein, degradation
    protein, ubiquitin dependent proteolysis
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling signal transduction
    Notch signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
    zinc Zn2+
    protein
  • binding to NUMB via its phosphotyrosine interacting domain
  • interacting with endogenous retrovirus K protein Np9
  • interacting with SPHKIP, and with SKI interacting protein (SKIP) via PDZ domains
  • interacting with ERBB2
  • interaction of CSK with LNX1 depends on the C-terminal PDZ ligand of CSK
  • interacts with the cell fate determinant NUMBL, a Numb homolog-like gene involved in the maintenance of neural progenitor cells during embryonic neurogenesis
  • is a protein binding to claudin-1 (promoted polyubiquitylation of claudins)
  • potential binding partner for RHOC (LNX1, LNX2 and RHOC might be part of a larger protein complex that would have important functions in signaling transduction about regulating the transcriptional activities of AP-1)
  • interaction of RNASEL with the E3 ubiquitin ligase and scaffolding protein LNX1, a regulator of tight junction proteins
    • cell & other
    REGULATION
    Phosphorylated by CSK, but CSK itself is ubiquitinated by LNX1, suggesting an interdependent regulation of CSK and LNX1
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --low  
    in 100p100 of gliomas including low- and high-grade ones
    constitutional     --low  
    could result in cell cycle arrest in G0/G1 phase through inhibition of CTNNB, MAPK, NFKB, MYC-dependent pathway and activation of TP53, TGFB-dependent pathway
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target might act as a diagnostic marker and a potential therapeutic target for glioma
    ANIMAL & CELL MODELS