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lamin-B receptor; integral nuclear envelope inner membrane protein; LMN2R (LBR)
Function:
- anchors the lamina & the heterochromatin to the inner nuclear membrane
- interacts directly with CBX5
- can interact with chromodomain proteins
- interacts directly with DNA
- interaction with DNA is sequence independent with higher affinity for supercoiled & relaxed circular DNA than linear DNA
- phosphorylated by CDC2 protein kinase in mitosis when the inner nuclear membrane breaks down into vesicles that dissociate from the lamina & the chromatin.
- phosphorylated by different protein kinases in interphase when the membrane is associated with these structures
- phosphorylation of LBR & HP1 proteins may be responsible for some of the alterations in chromatin organization & nuclear structure which occur at various times during the cell cycle
Structure: belongs to the ERG4/ERG24 family
Compartment: nucleus inner membrane
Pathology:
- defects in LBR are a cause of
a) Pelger-Huet anomaly
b) Greenberg skeletal dysplasia
General
nuclear protein
phosphoprotein
receptor
transmembrane 8 protein
Properties
SIZE: entity length = 615 aa
MW = 71 kD
COMPARTMENT: cell nucleus
MOTIF: Nucleoplasmic {1-208}
MOTIF: Ser phosphorylation site {S99}
Thr phosphorylation site {T118}
transmembrane domain {212-232}
transmembrane domain {258-278}
transmembrane domain {299-319}
transmembrane domain {326-346}
transmembrane domain {386-406}
transmembrane domain {447-467}
transmembrane domain {481-501}
transmembrane domain {561-581}
Database Correlations
OMIM correlations
UniProt Q14739
Pfam PF01222
Entrez Gene
Kegg hsa:3930
References
- UniProt :accession Q14739
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=LBR