Back to your search result

Description

Lamin-B receptor (UniProt: Q14739, also known as Integral nuclear envelope inner membrane protein, LMN2R) is encoded by the LBR gene (Gene ID: 3930) in human. Lamin-B receptor is a multi-pass membrane protein that anchors the lamina and the heterochromatin to the inner nuclear membrane. It is an integral membrane protein of the interphase nuclear envelope (NE). It contains a Tudor damain that lies within the first 62 amino acids. Its N-terminal end resides in the nucleoplasm, binding to lamin B and heterochromatin and the C-terminal end resides within the inner nuclear membrane, retreating with the endoplasmic reticulum (ER) away from condensing chromosomes during mitotic NE breakdown. During mitosis, Lamin-B receptor is retained within the retreating ER and kept out of the way of the mitotic apparatus. Lamin- B receptor is suggested to perform three vital functions in the cell: (a) contributes to post-mitotic nuclear reformation, (b) contributes to interphase NE growth, (c) contributes to compartmentalization of heterochromatin within the interphase nucleus. Lamin-B receptor is phosphorylated by Cdk1 in mitosis when the inner nuclear membrane breaks down into vesicles that dissociate from the lamina and the chromatin. It is phosphorylated by different protein kinases in interphase when the membrane is associated with these structures. Mutations in LBR gene are known to cause Pelger-Huet anomaly that is characterized by skeletal anomalies, developmental delay, and seizures. Some mutations cause Greenberg dysplasia that can lead to in utero lethality. (Ref.: Olins, AL., et al. (2010). Nucleus 1(1), 53-70).