Perturbation of wild-type lamin A metabolism results in a progeroid phenotype

Aging Cell

Volumn 7, Issue 3, 2008, Pages 355-367

  Candelario, Jose ^a   Sudhakar, Sivasubramaniam ^a   Navarro, Sonia ^a   Reddy, Sita ^b   Comai, Lucio ^a  

^a UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^b UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

Author keywords

Cell nucleus; Cellular senescence; Human; Longevity; Senescence

Indexed keywords

LAMIN A; LAMIN C; METALLOPROTEINASE; PROTEIN FARNESYLTRANSFERASE INHIBITOR;

ADOLESCENT; ADULT; AGED; APOPTOSIS; ARTICLE; CARBOXY TERMINAL SEQUENCE; CELL AGING; CELL NUCLEUS MEMBRANE; CHILD; CONTROLLED STUDY; GENE DELETION; GENE MUTATION; GENE OVEREXPRESSION; GROWTH DISORDER; HUMAN; HUMAN CELL; LIFESPAN; MEMBRANE DAMAGE; NEWBORN; PATHOGENESIS; PHENOTYPE; PRIORITY JOURNAL; PROGERIA; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN METABOLISM; WILD TYPE;

ADOLESCENT; ADULT; AGED, 80 AND OVER; APOPTOSIS; CELL AGING; CELL NUCLEUS; CELLS, CULTURED; GENETIC VECTORS; HUMANS; INFANT, NEWBORN; LAMIN TYPE A; MEMBRANE PROTEINS; METALLOENDOPEPTIDASES; MUTATION; PHENOTYPE; PROGERIA;

EID: 43449107982     PISSN: 14749718     EISSN: 14749726     Source Type: Journal    
DOI: 10.1111/j.1474-9726.2008.00393.x     Document Type: Article

References (19)

1. Ackerman J, Gilbert-Barness E (2002) Hutchinson-Gilford progeria syndrome: A pathologic study. Pediatr. Pathol. Mol. Med. 21, 1-13.
2. Agarwal AK, Fryns JP, Auchus RJ, Garg A (2003) Zinc metalloproteinase, ZMPSTE24, is mutated in mandibuloacral dysplasia. Hum. Mol. Genet. 12, 1995-2001.
3. Bergo MO, Gavino B, Ross J, Schmidt WK, Hong C, Kendall LV, Mohr A, Meta M, Genant H, Jiang Y, Wisner ER, Van Bruggen N, Carano RA, Michaelis S, Griffey SM, Young SG (2002) Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect. Proc. Natl Acad. Sci. USA 99, 13049-13054.
4. Bridger JM, Kill IR (2004) Aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis. Exp. Gerontol. 39, 717-724.
5. Capell BC, Erdos MR, Madigan JP, Fiordalisi JJ, Varga R, Conneely KN, Gordon LB, Der CJ, Cox AD, Collins FS (2005) Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome. Proc. Natl Acad. Sci. USA 102, 12879-12884.
6. Comai L, Zomerdijk JC, Beckmann H, Zhou S, Admon A, Tjian R (1994) Reconstitution of transcription factor SL1: Exclusive binding of TBP by SL1.or TFIID subunits. Science 266, 1966-1972.
7. De Sandre-Giovannoli A, Bernard R, Cau P, Navarro C, Amiel J, Boccaccio I, Lyonnet S, Stewart CL, Munnich A, Le Merrer M, Levy N (2003) Lamin a truncation in Hutchinson-Gilford progeria. Science 300, 2055.
8. Dimri GP, Campisi J (1994) Molecular and cell biology of replicative senescence. Cold Spring Harb. Symp. Quant. Biol. 59, 67-73.
9. Eriksson M, Brown WT, Gordon LB, Glynn MW, Singer J, Scott L, Erdos MR, Robbins CM, Moses TY, Berglund P, Dutra A, Pak E, Durkin S, Csoka AB, Boehnke M, Glover TW, Collins FS (2003) Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome. Nature 423, 293-298.
10. Glynn MW, Glover TW (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.
11. Goldman RD, Shumaker DK, Erdos MR, Eriksson M, Goldman AE, Gordon LB, Gruenbaum Y, Khuon S, Mendez M, Varga R, Collins FS (2004) Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome. Proc. Natl Acad. Sci. USA 101, 8963-8968.
12. Li B, Navarro S, Kasahara N, Comai L (2004) Identification and biochemical characterization of a Werner's syndrome protein complex with Ku70/80 and poly (ADP-ribose) polymerase-1. J. Biol. Chem. 279, 13659-13667.
13. Mallampalli MP, Huyer G, Bendale P, Gelb MH, Michaelis S (2005) Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome. Proc. Natl Acad. Sci. USA 102, 14416-14421.
14. Navarro CL, Cadinanos J, De Sandre-Giovannoli A, Bernard R, Courrier S, Boccaccio I, Boyer A, Kleijer WJ, Wagner A, Giuliano F, Beemer FA, Freije JM, Cau P, Hennekam RC, Lopez-Otin C, Badens C, Levy N (2005) Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors. Hum. Mol. Genet. 14, 1503-1513.
15. Pendas AM, Zhou Z, Cadinanos J, Freije JM, Wang J, Hultenby K, Astudillo A, Wernerson A, Rodriguez F, Tryggvason K, Lopez-Otin C (2002) Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase-deficient mice. Nat. Genet. 31, 94-99.
16. Pollex RL, Hegele RA (2004) Hutchinson-Gilford progeria syndrome. Clin. Genet. 66, 375-381.
17. Scaffidi P, Misteli T (2006) Lamin A-dependent nuclear defects in human aging. Science 312, 1059-1063.
18. Toth JI, Yang SH, Qiao X, Beigneux AP, Gelb MH, Moulson CL, Miner JH, Young SG, Fong LG (2005) Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes. Proc. Natl Acad. Sci. USA 102, 12873-12878.
19. Yang SH, Bergo MO, Toth JI, Qiao X, Hu Y, Sandoval S, Meta M, Bendale P, Gelb MH, Young SG, Fong LG (2005) Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation. Proc. Natl Acad. Sci. USA 102, 10291-10296.