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FLASH GENE
Symbol FBLIM1 contributors: mct - updated : 26-06-2017
HGNC name filamin binding LIM protein 1
HGNC id 24686
Corresponding disease
CRMO2 chronic recurrent multifocal osteomyelitis 2
Location 1p36.21      Physical location : 16.085.254 - 16.113.084
Synonym name
  • migfilin
  • mitogen-inducible 2-interacting protein
  • CSX-associated LIM
    • Synonym symbol(s) FBLP1, CAL, FBLP-1, DKFZp434G171, RP11-169K16.5
    DNA
    TYPE functioning gene
    STRUCTURE 27.83 kb     9 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 splicing 3363 40.5 373 heart, kidney, lung, pancreas, placenta and platelets 2005 15671069
  • interacts with PLEKHC1, FLNC, FLNA
  • recruited and localized at actin stress fibers and clustered at cell-ECM adhesion sites through interaction with PLEKHC1
    • 6 splicing 1521 40 374 brain, heart, kidney, lung, pancreas, placenta, skeletal muscle and platelets 2005 15671069
  • interacts with FLNB, NKX2-5
  • localized at actin stress fibers
    • 5 splicing 2793 30.6 276 heart, kidney, lung, pancreas, placenta and platelets 2005 15671069
  • interacts with PLEKHC1, FLNA, FLNC
  • recruited and localized at actin stress fibers and clustered at cell-ECM adhesion sites through interaction with PLEKHC1
    • - - 3515 - 373 - 2005 15671069
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Cardiovascularendothelial cell
    not specificepithelial cell
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a proline-rich domain near its N terminus, with two clusters of proline-rich sequences; first located at positions 86–112, mediating the interaction with the vasodilator-stimulated phosphoprotein (VASP) EVH1 domain, the second encompassing AAs 140–170 (Zhao 2009)
  • three LIM domain
  • C-terminal LIM domains of migfilin that dictate its Focal adhesions (FAs) localization
    • HOMOLOGY
    interspecies homolog to murine Fblim1 (78.8pc)
    homolog to rattus Cal (79.0pc)
    Homologene
    FAMILY
    CATEGORY adaptor , adhesion
    SUBCELLULAR LOCALIZATION     plasma membrane,junction,adherens
        intracellular
    intracellular,cytoplasm,cytoskeleton
    intracellular,nucleus
    text
  • localized at cell junctions and also in the nucleus
  • associated with actin bundles bridging neighboring cells
    • basic FUNCTION
  • required for the cell shape modulation and regulating actin remodeling and cell morphology
  • may link cell adhesion structures to the actin cytoskeleton
  • adaptor protein, functioning as an important activator of Src, linking cell-ECM adhesion to Src activation and survival signaling (Zhao 2009)
  • may affect cardiomyocyte differentiation after binding with the CSX/NKX2-5 transcription factor
  • important role in cell-ECM adhesion-mediated regulation of Src activation and protection against anoikis (Zhao 2009)
  • acts as a molecular switch to disconnect filamin from integrin for regulating integrin activation and dynamics of extracellular matrix-actin linkage (Ithychanda 2009)
  • regulates bone remodeling through modulating both osteoblast behavior and osteoclast differentiation
  • kindlin- and filamin-binding focal adhesion protein, essential for proper control of bone remodeling
  • FBLIM1 may work in concert with its binding partners FERMT2 and filamin in bone
  • is critical for cell shape and motile regulation
  • role of FBLIM1 in Osteoarthritis (OA), suggesting the importance of cell-ECM adhesion proteins in OA pathogenesis
  • novel cell-matrix adhesion protein known to interact with VASP and be localized both at cell-matrix and cell-cell adhesions
    • CELLULAR PROCESS cell life, differentiation
    cell migration & motility
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
  • FBLIM1-mediated signaling pathway is dysfunctional in multiple types of carcinoma cells, which likely contributes to aberrant Src activation and anoikis resistance in these cancerous cells (Zhao 2009)
  • SRC, FERMT2 and FBLIM1 together constitute a positive feedback loop that controls SRC activity and regulates integrin-mediated cellular functions
    • a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with filamin B and FERMT2
  • interacting with VASP and regulating VASP localization to cell matrix adhesions and migration (Zhang 2006)
  • interacts with not only Src SH3 domain but also Src SH2 domain, albeit the latter interaction is weaker than the former (Zhao 2009)
  • FLNA can act broadly as an inhibitor and FBLIM1 is a promoter of integrin activation
  • FERMT2 and filamin, which directly bind FBLIM1, have been implicated in regulation of bone remodeling
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) CRMO2
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional        
    increased cytoplasmic level of migfilin is associated with higher grades of human leiomyosarcoma (Papachristou 2007)
    tumoral     --over  
    is associated with poor prognosis for patients with glioma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
  • use of FBLIM1 as a molecular marker in glioma for early diagnosis and as an indicator of prognosis
    • Therapy target therapeutic intervention targeting the migfilin signaling pathway may provide a novel approach to restore anoikis sensitivity and alleviate cancer progression (Zhao 2009)
    SystemTypeDisorderPubmed
    cancerdigestiveliver
    is a potential therapeutic target against hepatocellular carcinoma metastasis (PMID:25773778)
    cancer  
    therapeutic intervention targeting the migfilin signaling pathway may provide a novel approach to restore anoikis sensitivity and alleviate cancer progression
    ANIMAL & CELL MODELS
  • inactivation of Fblim1 (the gene encoding Fblp-1) in mice resulted in a severe osteopenic phenotype