Progeria, the nucleolus and farnesyltransferase inhibitors

Biochemical Society Transactions

Volumn 38, Issue 1, 2010, Pages 287-291

  Mehta, Ishita S ^a   Bridger, Joanna M ^a   Kill, Ian R ^a  

^a BRUNEL UNIVERSITY   (United Kingdom)

Author keywords

Farnesyltransferase I inhibitor; Fibrillarin; Hutchinson Gilford progeria syndrome (HGPS); Ki67; Nucleolus

Indexed keywords

LAMIN A; N [[5 [(2 AMINO 3 MERCAPTOPROPYL)AMINO][1,1' BIPHENYL] 2 YL]CARBONYL]METHIONINE METHYL ESTER; NUCLEAR PROTEIN; PROGERIN; PROTEIN FARNESYLTRANSFERASE; PROTEIN FARNESYLTRANSFERASE INHIBITOR; UNCLASSIFIED DRUG;

ARTICLE; CELL STRUCTURE; CLINICAL FEATURE; DRUG MECHANISM; DRUG PROTEIN BINDING; HUMAN; NONHUMAN; NUCLEAR LAMINA; NUCLEOLUS; POINT MUTATION; PRIORITY JOURNAL; PROGERIA; PROTEIN FUNCTION; PROTEIN PROCESSING;

CELL NUCLEOLUS; CHILD; CHROMOSOMAL PROTEINS, NON-HISTONE; CLINICAL TRIALS AS TOPIC; ENZYME INHIBITORS; FARNESYLTRANSTRANSFERASE; HUMANS; LAMIN TYPE A; METHIONINE; PROGERIA;

EID: 76449118151     PISSN: 03005127     EISSN: 14708752     Source Type: Journal    
DOI: 10.1042/BST0380287     Document Type: Article

References (49)

1. Worman, H.J., Fong, L.G., Muchir, A. and Young, S.G. (2009) Laminopathies and the long strange trip from basic cell biology to therapy. J. Clin. Invest. 119, 1825-1836
2. De Sandre-Giovannoli, A., Bernard, R., Cau, P., Navarro, C., Amiel, J., Boccaccio, I., Lyonnet, S., Stewart, C.L., Munnich, A., Le Merrer, M. and Levy, N. (2003) Lamin a truncation in Hutchinson-Gilford progeria. Science 300, 2055
3. Eriksson, M., Brown, W.T., Gordon, L.B., Glynn, M.W., Singer, J., Scott, L., Erdos, M.R., Robbins, C.M., Moses, T.Y., Berglund, P. et al. (2003) Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome. Nature 423, 293-298
4. Dechat, T., Pfleghaar, K., Sengupta, K., Shimi, T., Shumaker, D.K., Solimando, L. and Goldman, R.D. (2008) Nuclear lamins: major factors in the structural organization and function of the nucleus and chromatin. Genes Dev. 22, 832-853
5. Fong, L.G., Vickers, T.A., Farber, E.A., Choi, C., Yun, U.J., Hu, Y., Yang, S.H., Coffinier, C., Lee, R., Yin, L. et al. (2009) Activating the synthesis of progerin, the mutant prelamin A in Hutchinson-Gilford progeria syndrome, with antisense oligonucleotides. Hum. Mol. Genet. 18, 2462-2471
6. Shimi, T., Pfleghaar, K., Kojima, S., Pack, C.G., Solovei, I., Goldman, A.E., Adam, S.A., Shumaker, D.K., Kinjo, M., Cremer, T. and Goldman, R.D. (2008) The A- and B-type nuclear lamin networks: microdomains involved in chromatin organization and transcription. Genes Dev. 22, 3409-3421
7. Meaburn, K.J., Cabuy, E., Bonne, G., Levy, N., Morris, G.E., Novelli, G., Kill, I.R. and Bridger, J.M. (2007) Primary laminopathy fibroblasts display altered genome organization and apoptosis. Aging Cell 6, 139-153
8. Beck, L.A., Hosick, T.J. and Sinensky, M. (1990) Isoprenylation is required for the processing of the lamin A precursor. J. Cell Biol. 110, 1489-1499
9. Hennekes, H. and Nigg, E.A. (1994) The role of isoprenylation in membrane attachment of nuclear lamins. A single point mutation prevents proteolytic cleavage of the lamin A precursor and confers membrane binding properties. J. Cell Sci. 107, 1019-1029
10. Pendas, A.M., Zhou, Z., Cadinanos, J., Freije, J.M., Wang, J., Hultenby, K., Astudillo, A., Wernerson, A., Rodriguez, F., Tryggvason, K. and Lopez-Otin, C. (2002) Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase-deficient mice. Nat. Genet. 31, 94-99
11. Broers, J.L., Ramaekers, F.C., Bonne, G., Yaou, R.B. and Hutchison, C.J. (2006) Nuclear lamins: laminopathies and their role in premature ageing. Physiol. Rev. 86, 967-1008
12. Capell, B.C., Erdos, M.R., Madigan, J.P., Fiordalisi, J.J., Varga, R., Conneely, K.N., Gordon, L.B., Der, C.J., Cox, A.D. and Collins, F.S. (2005) Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome. Proc. Natl. Acad. Sci. U.S.A. 102, 12879-12884
13. Young, S.G., Meta, M., Yang, S.H. and Fong, L.G. (2006) Prelamin A farnesylation and progeroid syndromes. J. Biol. Chem. 281, 39741-39745
14. Goldman, R.D., Shumaker, D.K., Erdos, M.R., Eriksson, M., Goldman, A.E., Gordon, L.B., Gruenbaum, Y., Khuon, S., Mendez, M., Varga, R. and Collins, F.S. (2004) Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome. Proc. Natl. Acad. Sci. U.S.A. 101, 8963-8968
15. Scaffidi, P. and Misteli, T. (2005) Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome. Nat. Med. 11, 440-445
16. Adjei, A.A. (2005) Farnesyltransferase inhibitors. Cancer Chemother. Biol. Response Modif. 22, 123-133
17. Glynn, M.W. and Glover, T.W. (2005) Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition. Hum. Mol. Genet. 14, 2959-2969
18. Mallampalli, M.P., Huyer, G., Bendale, P., Gelb, M.H. and Michaelis, S. (2005) Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome. Proc. Natl. Acad. Sci. U.S.A. 102, 14416-14421
19. Toth, J.I., Yang, S.H., Qiao, X., Beigneux, A.P., Gelb, M.H., Moulson, C.L., Miner, J.H., Young, S.G. and Fong, L.G. (2005) Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes. Proc. Natl. Acad. Sci. U.S.A. 102, 12873-12878
20. Yang, S.H., Bergo, M.O., Toth, J.I., Qiao, X., Hu, Y., Sandoval, S., Meta, M., Bendale, P., Gelb, M.H., Young, S.G. and Fong, L.G. (2005) Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation. Proc. Natl. Acad. Sci. U.S.A. 102, 10291-10296
21. Verstraeten, V.L., Ji, J.Y., Cummings, K.S., Lee, R.T. and Lammerding, J. (2008) Increased mechanosensitivity and nuclear stiffness in Hutchinson-Gilford progeria cells: effects of farnesyltransferase inhibitors. Aging Cell 7, 383-393
22. Liu, Y., Rusinol, A., Sinensky, M., Wang, Y. and Zou, Y. (2006) DNA damage responses in progeroid syndromes arise from defective maturation of prelamin A. J. Cell Sci. 119, 4644-4649
23. Fong, L.G., Frost, D., Meta, M., Qiao, X., Yang, S.H., Coffinier, C. and Young, S.G. (2006) A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria. Science 311, 1621-1623
24. Yang, S.H., Meta, M., Qiao, X., Frost, D., Bauch, J., Coffinier, C., Majumdar, S., Bergo, M.O., Young, S.G. and Fong, L.G. (2006) A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation. J. Clin. Invest. 116, 2115-2121
25. Yang, S.H., Qiao, X., Farber, E., Chang, S.Y., Fong, L.G. and Young, S.G. (2008) Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele. J. Biol. Chem. 283, 7094-7099
26. Capell, B.C., Olive, M., Erdos, M.R., Cao, K., Faddah, D.A., Tavarez, U.L., Conneely, K.N., Qu, X., San, H., Ganesh, S.K. et al. (2008) A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model. Proc. Natl. Acad. Sci. U.S.A. 105, 15902-15907
27. Kieran, M.W., Gordon, L. and Kleinman, M. (2007) New approaches to progeria. Pediatrics 120, 834-841
28. Gordon, L.B., Harling-Berg, C.J. and Rothman, F.G. (2008) Highlights of the 2007 Progeria Research Foundation scientific workshop: progress in translational science. J. Gerontol. A Biol. Sci. Med. Sci. 63, 777-787
29. Goldman, A.E., Moir, R.D., Montag-Lowy, M., Stewart, M. and Goldman, R.D. (1992) Pathway of incorporation of microinjected lamin A into the nuclear envelope. J. Cell Biol. 119, 725-735
30. Barboro, P., D'Arrigo, C., Diaspro, A., Mormino, M., Alberti, I., Parodi, S., Patrone, E. and Balbi, C. (2002) Unraveling the organization of the internal nuclear matrix: RNA-dependent anchoring of NuMA to a lamin scaffold. Exp. Cell Res. 279, 202-218
31. Hozak, P., Sasseville, A.M., Raymond, Y. and Cook, P.R. (1995) Lamin proteins form an internal nucleoskeleton as well as a peripheral lamina in human cells. J. Cell Sci. 108, 635-644
32. Bridger, J.M., Kill, I.R., O'Farrell, M. and Hutchison, C.J. (1993) Internal lamin structures within G1 nuclei of human dermal fibroblasts. J. Cell. Sci. 104, 297-306
33. Kennedy, B.K., Barbie, D.A., Classon, M., Dyson, N. and Harlow, E. (2000) Nuclear organization of DNA replication in primary mammalian cells. Genes Dev. 14, 2855-2868
34. Moir, R.D., Montag-Lowy, M. and Goldman, R.D. (1994) Dynamic properties of nuclear lamins: lamin B is associated with sites of DNA replication. J. Cell Biol. 125, 1201-1212
35. Jagatheesan, G., Thanumalayan, S., Muralikrishna, B., Rangaraj, N., Karande, A.A. and Parnaik, V.K. (1999) Colocalization of intranuclear lamin foci with RNA splicing factors. J. Cell Sci. 112, 4651-4661
36. Martin, C., Chen, S., Maya-Mendoza, A., Lovric, J., Sims, P.F. and Jackson, D.A. (2009) Lamin B1 maintains the functional plasticity of nucleoli. J. Cell Sci. 122, 1551-1562
37. Jordan, E.G. (1984) Nucleolar nomenclature. J. Cell Sci. 67, 217-220
38. Hernandez-Verdun, D. (1991) The nucleolus today. J. Cell Sci. 99, 465-471
39. r nucleolar antigen specific for the dense fibrillar component of the nucleoli. J. Cell Sci. 95, 371-381
40. Kill, I.R. (1996) Localisation of the Ki-67 antigen within the nucleolus: evidence for a fibrillarin-deficient region of the dense fibrillar component. J. Cell Sci. 109, 1253-1263
41. Spector, D.L., Ochs, R.L. and Busch, H. (1984) Silver staining, immunofluorescence, and immunoelectron microscopic localization of nucleolar phosphoproteins B23 and C23. Chromosoma 90, 139-148
42. Schmidt-Zachmann, M.S., Hugle-Dorr, B. and Franke, W.W. (1987) A constitutive nucleolar protein identified as a member of the nucleoplasmin family. EMBO J. 6, 1881-1890
43. Guldner, H.H., Szostecki, C., Vosberg, H.P., Lakomek, H.J., Penner, E. and Bautz, F.A. (1986) Scl 70 autoantibodies from scleroderma patients recognize a 95 kDa protein identified as DNA topoisomerase I. Chromosoma 94, 132-138
44. Reimer, G., Rose, K.M., Scheer, U. and Tan, E.M. (1987) Autoantibody to RNA polymerase I in scleroderma sera. J. Clin. Invest. 79, 65-72
45. Boisvert, F.M., van Koningsbruggen, S., Navascues, J. and Lamond, A.I. (2007) The multifunctional nucleolus. Nat. Rev. Mol. Cell Biol. 8, 574-585
46. Mongelard, F. and Bouvet, P. (2007) Nucleolin: a multiFACeTed protein. Trends Cell Biol. 17, 80-86
47. Ochs, R.L., Lischwe, M.A., Spohn, W.H. and Busch, H. (1985) Fibrillarin: a new protein of the nucleolus identified by autoimmune sera. Biol. Cell 54, 123-133
48. Fomproix, N., Gebrane-Younes, J. and Hernandez-Verdun, D. (1998) Effects of anti-fibrillarin antibodies on building of functional nucleoli at the end of mitosis. J. Cell Sci. 111, 359-372
49. Bridger, J.M. and Kill, I.R. (2004) Aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis. Exp. Gerontol. 39, 717-724