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Gene Review

LBR  -  lamin B receptor

Homo sapiens

Synonyms: DHCR14B, Integral nuclear envelope inner membrane protein, LMN2R, Lamin-B receptor, PHA, ...
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Disease relevance of LBR

  • Heterozygous mutations in LBR cause Pelger-Huët anomaly, characterized by morphologically abnormal neutrophil nuclei, and homozygous mutations cause HEM/Greenberg skeletal dysplasia, characterized by developmental abnormalities and 3 beta-hydroxysterol-epsilon-14-reductase deficiency [1].
  • A fusion protein containing the amino-terminal domain of human LBR is recognized by autoantibodies from patients with primary biliary cirrhosis, and these serum antibodies label the nuclear envelope when examined by immunofluorescence microscopy [2].
  • These results indicate that dissociation of HP1alpha from LBR and consequent perturbation of the nuclear envelope induced by polyomavirus Agno promote the translocation of virions out of the nucleus [3].
  • Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly [4].
  • Serum immunoglobulins, salivary IgA, natural isohaemagglutinins, Fc-IgG receptor-bearing cells and suppressor T lymphocytes were absent, together with an impaired de novo DNA synthesis after PHA, Con A, PWM and Cowan I strain from Staphylococcus aureus stimulation [5].

Psychiatry related information on LBR

  • This was confirmed by time studies where 1) a 2 min HIV-pretreatment followed by washing before stimulation was sufficient to inhibit PHA induced proliferation of normal lymphocytes, and 2) addition of HIV to PHA prestimulated lymphocytes failed to inhibit proliferation, e.g., there was no effect on preactivated lymphocytes [6].
  • The PHA-induced calcium signal in lymphocytes is altered after blockade of K(+)-channels in Alzheimer's disease [7].
  • High responders to life stress had significantly less response to PHA, and high responders to cold pressor stress had lower PWM responses [8].
  • An analysis of all aphidicolin-inducible breakpoints has been carried out in PHA stimulated T-lymphocytes of five patients with classical Rett syndrome, their mothers and a group of age matched controls [9].
  • The only difference in the immunological status of the patients who had experienced a stressful life event in the previous twelve months and those that had not, involved PHA mitogenic activity which was significantly lower after a stressful event [10].

High impact information on LBR


Chemical compound and disease context of LBR

  • The antiviral activity of azidothymidine (AZT), dideoxycytidine (ddC), and dideoxyinosine (ddI) against HIV-1 was comparatively evaluated in PHA-stimulated PBM [13].
  • The percent change achieved by the addition of indomethacin to a PHA-induced proliferative response was significantly increased in patients with cirrhosis compared with controls (p less than 0.001) [14].
  • Blastogen A induced peak uptake of thymidine during day 6 of tissue culture whereas PHA induced peak uptake during day 5 and tetanus during day 8 [15].
  • Cell-to-cell fusion studies showed significantly attenuated fusion between freshly isolated resting T cells and T cells constitutively expressing high levels of HIV envelope glycoprotein (HXB/gpt) compared with T cells first stimulated with either PHA or OKT3 mAb [16].
  • This antiserum was found to block U50,488H-mediated inhibition of 1) Staphylococcus aureus Cowen strain I-induced B and T lymphocyte proliferation, 2) PHA-induced T lymphocyte proliferation, and 3) S. aureus Cowen strain I-induced IgG production [17].

Biological context of LBR


Anatomical context of LBR


Associations of LBR with chemical compounds

  • 1. LBR has a nucleoplasmic, amino-terminal domain of approximately 200 amino acids followed by a carboxyl-terminal domain similar in sequence to yeast and plant sterol reductases [18].
  • To test the effects of C14 reductase inhibitors on LBR activity, we constructed EMY54, an ergosterol-requiring strain that is devoid of both sterol C8-C7 isomerase and sterol C14 reductase activities [25].
  • Sterol biosynthesis and proliferation of LBR-producing cells were found to be highly susceptible to fenpropimorph and tridemorph, but only moderately susceptible to SR 31747 [25].
  • The heterologous LBR was not extracted by 8 M urea, indicating that it was integrated into the membrane [26].
  • Participation of multiple kinases in regulation of the binding of lamin B receptor (LBR) to chromatin was suggested previously (Takano, M., Takeuchi, M., Ito, H., Furukawa, K., Sugimoto, K., Omata, S., and Horigome, T. (2002) Eur. J. Biochem. 269, 943-953) [27].

Physical interactions of LBR


Other interactions of LBR

  • Among the candidate genes to consider for the non-LMNA-associated forms of FPLD are other components of the inner nuclear membrane, such as lamin B1 and B2 and the lamin B receptor [20].
  • Cross-linking experiments, however, illustrate a tight association of HA95 with LBR and LAP2 only [30].
  • The modular domain organization of HP1-type proteins and LBR can explain some of the diverse protein-protein interactions at the chromatin-lamina-membrane interface of the nuclear envelope [31].
  • We also define a new class of variant PxVxL CSD-binding motifs in Sp100A, LBR, and ATRX [32].
  • Interestingly, emerin and LBR initially accumulated at distinct, separate locations, but then became uniform 8 minutes after the onset of anaphase, concurrent with the recovery of nuclear import function [33].

Analytical, diagnostic and therapeutic context of LBR


  1. Components of the nuclear envelope and their role in human disease. Worman, H.J. Novartis Found. Symp. (2005) [Pubmed]
  2. Primary structure analysis and lamin B and DNA binding of human LBR, an integral protein of the nuclear envelope inner membrane. Ye, Q., Worman, H.J. J. Biol. Chem. (1994) [Pubmed]
  3. Dissociation of heterochromatin protein 1 from lamin B receptor induced by human polyomavirus agnoprotein: role in nuclear egress of viral particles. Okada, Y., Suzuki, T., Sunden, Y., Orba, Y., Kose, S., Imamoto, N., Takahashi, H., Tanaka, S., Hall, W.W., Nagashima, K., Sawa, H. EMBO Rep. (2005) [Pubmed]
  4. Mutations at the mouse ichthyosis locus are within the lamin B receptor gene: a single gene model for human Pelger-Huët anomaly. Shultz, L.D., Lyons, B.L., Burzenski, L.M., Gott, B., Samuels, R., Schweitzer, P.A., Dreger, C., Herrmann, H., Kalscheuer, V., Olins, A.L., Olins, D.E., Sperling, K., Hoffmann, K. Hum. Mol. Genet. (2003) [Pubmed]
  5. Improvement of natural killer activity and of T cells after thymopoietin pentapeptide therapy in a patient with severe combined immunodeficiency. Fiorilli, M., Sirianni, M.C., Pandolfi, F., Quinti, I., Tosti, U., Aiuti, F., Goldstein, G. Clin. Exp. Immunol. (1981) [Pubmed]
  6. HIV inhibits the early steps of lymphocyte activation, including initiation of inositol phospholipid metabolism. Hofmann, B., Nishanian, P., Baldwin, R.L., Insixiengmay, P., Nel, A., Fahey, J.L. J. Immunol. (1990) [Pubmed]
  7. The PHA-induced calcium signal in lymphocytes is altered after blockade of K(+)-channels in Alzheimer's disease. Bondy, B., Hofmann, M., Müller-Spahn, F., Witzko, M., Hock, C. Journal of psychiatric research. (1996) [Pubmed]
  8. Effects of psychophysical stress on surgical outcome. Linn, B.S., Linn, M.W., Klimas, N.G. Psychosomatic medicine. (1988) [Pubmed]
  9. Additional clinical and cytogenetic findings associated with Rett syndrome. Simonic, I., Gericke, G.S., Lippert, M., Schoeman, J.F. Am. J. Med. Genet. (1997) [Pubmed]
  10. Stress antecedents and immune status in recently diagnosed type I (insulindependent) diabetes mellitus. Vialettes, B., Ozanon, J.P., Kaplansky, S., Farnarier, C., Sauvaget, E., Lassmann-Vague, V., Bernard, D., Vague, P. Diabète & métabolisme. (1989) [Pubmed]
  11. Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huët anomaly). Hoffmann, K., Dreger, C.K., Olins, A.L., Olins, D.E., Shultz, L.D., Lucke, B., Karl, H., Kaps, R., Müller, D., Vayá, A., Aznar, J., Ware, R.E., Sotelo Cruz, N., Lindner, T.H., Herrmann, H., Reis, A., Sperling, K. Nat. Genet. (2002) [Pubmed]
  12. DNA-dependent RNA polymerase levels during the response of human peripheral lymphocytes to phytohemagglutinin. Jaehning, J.A., Stewart, C.C., Roeder, R.G. Cell (1975) [Pubmed]
  13. Differential phosphorylation of azidothymidine, dideoxycytidine, and dideoxyinosine in resting and activated peripheral blood mononuclear cells. Gao, W.Y., Shirasaka, T., Johns, D.G., Broder, S., Mitsuya, H. J. Clin. Invest. (1993) [Pubmed]
  14. Studies on lymphocyte hyporesponsiveness in cirrhosis: the role of increased monocyte suppressor cell activity. Holdstock, G., Chastenay, B.F., Krawitt, E.L. Gastroenterology (1982) [Pubmed]
  15. Characterization of the human cellular immune response to purified group A streptococcal blastogen A1. Regelmann, W.E., Gray, E.D., Wannamaker, L.W. J. Immunol. (1982) [Pubmed]
  16. Evidence that T cell activation is required for HIV-1 entry in CD4+ lymphocytes. Gowda, S.D., Stein, B.S., Mohagheghpour, N., Benike, C.J., Engleman, E.G. J. Immunol. (1989) [Pubmed]
  17. Anti-human kappa opioid receptor antibodies: characterization of site-directed neutralizing antibodies specific for a peptide kappa R(33-52) derived from the predicted amino terminal region of the human kappa receptor. Buchner, R.R., Vogen, S.M., Fischer, W., Thoman, M.L., Sanderson, S.D., Morgan, E.L. J. Immunol. (1997) [Pubmed]
  18. The human lamin B receptor/sterol reductase multigene family. Holmer, L., Pezhman, A., Worman, H.J. Genomics (1998) [Pubmed]
  19. SRPK1 and LBR protein kinases show identical substrate specificities. Papoutsopoulou, S., Nikolakaki, E., Giannakouros, T. Biochem. Biophys. Res. Commun. (1999) [Pubmed]
  20. Single-nucleotide polymorphisms of the nuclear lamina proteome. Hegele, R.A., Yuen, J., Cao, H. J. Hum. Genet. (2001) [Pubmed]
  21. Chromosomal assignment of human nuclear envelope protein genes LMNA, LMNB1, and LBR by fluorescence in situ hybridization. Wydner, K.L., McNeil, J.A., Lin, F., Worman, H.J., Lawrence, J.B. Genomics (1996) [Pubmed]
  22. Nuclear envelope organization in papillary thyroid carcinoma. Fischer, A.H., Taysavang, P., Weber, C.J., Wilson, K.L. Histol. Histopathol. (2001) [Pubmed]
  23. Targeting of membranes to sea urchin sperm chromatin is mediated by a lamin B receptor-like integral membrane protein. Collas, P., Courvalin, J.C., Poccia, D. J. Cell Biol. (1996) [Pubmed]
  24. Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene. Waterham, H.R., Koster, J., Mooyer, P., Noort Gv, G., Kelley, R.I., Wilcox, W.R., Wanders, R.J., Hennekam, R.C., Oosterwijk, J.C. Am. J. Hum. Genet. (2003) [Pubmed]
  25. Human lamin B receptor exhibits sterol C14-reductase activity in Saccharomyces cerevisiae. Silve, S., Dupuy, P.H., Ferrara, P., Loison, G. Biochim. Biophys. Acta (1998) [Pubmed]
  26. Colocalization of vertebrate lamin B and lamin B receptor (LBR) in nuclear envelopes and in LBR-induced membrane stacks of the yeast Saccharomyces cerevisiae. Smith, S., Blobel, G. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  27. Regulation of binding of lamin B receptor to chromatin by SR protein kinase and cdc2 kinase in Xenopus egg extracts. Takano, M., Koyama, Y., Ito, H., Hoshino, S., Onogi, H., Hagiwara, M., Furukawa, K., Horigome, T. J. Biol. Chem. (2004) [Pubmed]
  28. Interaction between an integral protein of the nuclear envelope inner membrane and human chromodomain proteins homologous to Drosophila HP1. Ye, Q., Worman, H.J. J. Biol. Chem. (1996) [Pubmed]
  29. Characterization of p18, a component of the lamin B receptor complex and a new integral membrane protein of the avian erythrocyte nuclear envelope. Simos, G., Maison, C., Georgatos, S.D. J. Biol. Chem. (1996) [Pubmed]
  30. HA95 is a protein of the chromatin and nuclear matrix regulating nuclear envelope dynamics. Martins, S.B., Eide, T., Steen, R.L., Jahnsen, T., Skålhegg B, S., Collas, P. J. Cell. Sci. (2000) [Pubmed]
  31. Domain-specific interactions of human HP1-type chromodomain proteins and inner nuclear membrane protein LBR. Ye, Q., Callebaut, I., Pezhman, A., Courvalin, J.C., Worman, H.J. J. Biol. Chem. (1997) [Pubmed]
  32. The mammalian heterochromatin protein 1 binds diverse nuclear proteins through a common motif that targets the chromoshadow domain. Lechner, M.S., Schultz, D.C., Negorev, D., Maul, G.G., Rauscher, F.J. Biochem. Biophys. Res. Commun. (2005) [Pubmed]
  33. Live fluorescence imaging reveals early recruitment of emerin, LBR, RanBP2, and Nup153 to reforming functional nuclear envelopes. Haraguchi, T., Koujin, T., Hayakawa, T., Kaneda, T., Tsutsumi, C., Imamoto, N., Akazawa, C., Sukegawa, J., Yoneda, Y., Hiraoka, Y. J. Cell. Sci. (2000) [Pubmed]
  34. Expression and localization of nuclear proteins in autosomal-dominant Emery-Dreifuss muscular dystrophy with LMNA R377H mutation. Reichart, B., Klafke, R., Dreger, C., Krüger, E., Motsch, I., Ewald, A., Schäfer, J., Reichmann, H., Müller, C.R., Dabauvalle, M.C. BMC Cell Biol. (2004) [Pubmed]
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