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FLASH GENE
Symbol LBR contributors: mct/npt - updated : 05-03-2017
HGNC name lamin B receptor
HGNC id 6518
RNA
TRANSCRIPTS type messenger
text variant 1 and 2 encode the same protein
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
14 - 3806 - 615 - 2005 15882967
also called variant 1/isoform 1
14 - 3789 - 615 - 2005 15882967
also called variant 2/isoform 1
- - 1663 - 115 - 2005 15882967
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart    
Lymphoid/Immunelymph node   highly
 thymus   highly
Reproductivefemale systemuterus  highly
 male systemtestis  highly
Urinarykidney    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialbarrier/lining   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES Hydrophobic
STRUCTURE
motifs/domains
  • N terminal hydrophilic nucleoplasmic domain
  • a hydrophobic domain with eight transmembrane segments, projecting into the nucleoplasm and binding to both B-type lamins and chromatin proteins
  • first transmembrane domain promotes the LBR-bearing membrane production and the nuclear envelope expansion in interphase
  • a lamin B binding
  • a tudor (TD) domain (AAs 1-62) that primarily recognizes histone H4 lysine 20 dimethylation and is essential for chromatin compaction,
  • a whole nucleoplasmic region (AAs 1-211) is required for transcriptional repression , and TD/RS interface may potentially operate as a "histone chaperone-like platform"
  • a sterol reductase like C-terminal domain having two functional domains: a highly homologous to sterol reductases and encoding sterol 14-reductase activity, playing a critical role in cholesterol biosynthesis, process essential to both myeloid cell growth and functional maturation
  • HOMOLOGY
    Homologene
    FAMILY
  • LAP2 (lamina-associated protein 2) family
  • ERG4/ERG24 family
  • CATEGORY receptor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,organelle,membrane
    intracellular,nuclear envelope,int
    text
  • integral protein of the nuclear enveloppe inner membrane, binding the lamina to heterochromatin
  • integral membrane protein of the interphase nuclear envelope (NE)
  • basic FUNCTION
  • binding the lamina and the heterochromatin to the inner nuclear membrane
  • can interact with chromodomain proteins
  • bifunctional protein involved in nuclear organization and chromatin binding
  • playing an important role in targeting the nuclear envelope to chromatin in an importin beta-mediated process
  • integral nuclear envelope protein that interacts with chromatin and has homology to sterol reductases
  • possible mechanism involving PPP1CC and LBR for the regulation of nuclear membrane targeting to chromatin
  • recruited to chromatin through direct interaction with importin beta to contribute to the fusion of membrane vesicles and formation of the nuclear envelope
  • likely roles in post-mitotic nuclear reformation, interphase nuclear envelope growth and compartmentalization of nuclear architecture might have provided some evolutionary advantage to preservation of the LBR gene
  • polytopic protein of the nuclear envelope thought to connect the inner nuclear membrane with the underlying nuclear lamina and peripheral heterochromatin
  • plays a critical role in regulating myeloid differentiation
  • classical inner nuclear membrane protein that associates with heterochromatin
  • LMNA and LBR sequentially tether peripheral heterochromatin and inversely regulate differentiation
  • contributes to the functional integrity of the nucleus by tethering heterochromatin to the nuclear envelope
  • LBR possesses a modular structure and corresponding bifunctionality, acting in heterochromatin organization via its N-terminus and in cholesterol biosynthesis via its sterol-reductase C-terminal domain
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • constituent of nuclear inner membrane
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • binds both chromatin and lamin B as part of a meshwork of intermediate filament proteins known as the nuclear lamina
  • interacting with CBX5
  • interacting with CEBPE (transcriptional regulation of CEBPE, is necessary for morphological but not necessarily functional granulocyte maturation)
  • the N-terminal domain of LBR directly interacts with importin beta (KPNB1) in a Ran sensitive and importin alpha independent manner
  • MECP2 interacts with the inner nuclear membrane protein LBR and that the unstructured aminoacidic sequence linking the MBD and TRD domains of MECP2 is responsible for this association
  • targets the membrane precursor vesicles to chromatin by interacting with KPNB1 in a LBR phosphorylation-dependent manner during the NE assembly at the end of mitosis
  • nucleoporin AHCTF1, which is crucial for postmitotic NPC formation, is essential for recruiting the LBR to the chromosomal noncore region
  • depletion of AHCTF1 promoted LBR phosphorylation at the residues known to be phosphorylated by cyclin-dependent kinase (CDK) and serine/arginine protein kinases 1 and 2 (SRPK1 and SRPK2, respectively)
  • TMEM147 physically interacts with LBR, and the C-terminus of LBR is essential for their functional interaction
  • at the nuclear envelope, TMEM147 interacts with the C-terminal domain of the lamin B receptor (LBR), anchoring it to the inner membrane
  • cell & other
    REGULATION
    Phosphorylated by CDK1 (phosphorylation of LBR by CDK1 contributes to the temporal control)
    ASSOCIATED DISORDERS
    corresponding disease(s) PHA , HEMSK , RS
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    affects not only nuclear architecture but also proliferation, cell viability, and gene expression of hematopoietic cells
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS