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FLASH GENE
Symbol LMNB2 contributors: mct/npt - updated : 04-05-2017
HGNC name lamin B2
HGNC id 6638
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a N terminal head domain
  • a central rod domain
  • a nuclear localization signal (NLS)
  • a C terminal tail with a CAAX doamin (a farnesyl group is attached to the cystein residue of the &
  • 8722;CAAX box by a farnesyltransferase)Dechat 2008)
    mono polymer homomer , heteromer , tetramer
    HOMOLOGY
    interspecies homolog to rattus Lmnb2 (84.58 pc)
    homolog to murine Lmnb2 (83.53 pc)
    Homologene
    FAMILY
  • intermediate filament family, type V
  • CATEGORY structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,intermed filament
    intracellular,nuclear envelope,int
    intracellular,nuclear envelope,lamina
    basic FUNCTION
  • involved in nuclear stability, chromatin structure and gene expression
  • responsible of DNA replication origin
  • LMNA, LMNB1, LMNB2 not only constitute a scaffold for nuclear shape, rigidity and resistance to stress but also contribute to the organization of chromatin and chromosomal domains
  • LMNB2 interaction with MCM7 promotes non-small cell lung cancer (NSCLC) progression
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • coiled coil dimers associated to form a tetrameric subunit from which IFs assemble
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with TOP1, TOP2A in a cell cycle-modulated manner
  • lamin B2 binds to the C-terminus of MCM7, and competes with the binding of the tumor suppressor retinoblastoma (RB1) protein
  • cell & other
    REGULATION
    Other diassembly at the start and reassembly at the end of mitosis mediated by phosphorylation,dephosphorylation
    ASSOCIATED DISORDERS
    corresponding disease(s) LPPA , EPM9
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    in acquired partial lipodystrophy (Barraquer-Simons syndrome)
    constitutional germinal mutation      
    mutations will be identified in children with severe developmental brain disorders (Coffinier 2010)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
    Lmnb2&
    8722;/&
    8722; mice had a smaller cerebellum with a complete absence of foliation, and the hippocampus was also abnormal (Coffinier 2010)