PubMed

Reversible phenotype in a mouse model of Hutchinson-Gilford progeria syndrome.

Sagelius H, Rosengardten Y, Schmidt E, Sonnabend C, Rozell B, Eriksson M.

J Med Genet. 2008 Dec;45(12):794-801. Epub 2008 Aug 15.PMID: 18708427 [PubMed - indexed for MEDLINE]Related articles

Targeted transgenic expression of the mutation causing Hutchinson-Gilford progeria syndrome leads to proliferative and degenerative epidermal disease.

Sagelius H, Rosengardten Y, Hanif M, Erdos MR, Rozell B, Collins FS, Eriksson M.

J Cell Sci. 2008 Apr 1;121(Pt 7):969-78. Epub 2008 Mar 11.PMID: 18334552 [PubMed - indexed for MEDLINE]Related articlesFree article

Hutchinson-Gilford progeria syndrome.

Pollex RL, Hegele RA.

Clin Genet. 2004 Nov;66(5):375-81. Review.PMID: 15479179 [PubMed - indexed for MEDLINE]Related articles

Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition.

Glynn MW, Glover TW.

Hum Mol Genet. 2005 Oct 15;14(20):2959-69. Epub 2005 Aug 26.PMID: 16126733 [PubMed - indexed for MEDLINE]Related articlesFree article

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation.

Yang SH, Qiao X, Fong LG, Young SG.

Biochim Biophys Acta. 2008 Jan-Feb;1781(1-2):36-9. Epub 2007 Nov 26.PMID: 18082640 [PubMed - indexed for MEDLINE]Related articlesFree article

A progeroid syndrome in mice is caused by defects in A-type lamins.

Mounkes LC, Kozlov S, Hernandez L, Sullivan T, Stewart CL.

Nature. 2003 May 15;423(6937):298-301.PMID: 12748643 [PubMed - indexed for MEDLINE]Related articles

Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome.

Scaffidi P, Misteli T.

Nat Med. 2005 Apr;11(4):440-5. Epub 2005 Mar 6.PMID: 15750600 [PubMed - indexed for MEDLINE]Related articlesFree article

Hutchinson-Gilford progeria syndrome: clinical findings in three patients carrying the G608G mutation in LMNA and review of the literature.

Mazereeuw-Hautier J, Wilson LC, Mohammed S, Smallwood D, Shackleton S, Atherton DJ, Harper JI.

Br J Dermatol. 2007 Jun;156(6):1308-14. Epub 2007 Apr 25. Review.PMID: 17459035 [PubMed - indexed for MEDLINE]Related articles

Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome.

Capell BC, Erdos MR, Madigan JP, Fiordalisi JJ, Varga R, Conneely KN, Gordon LB, Der CJ, Cox AD, Collins FS.

Proc Natl Acad Sci U S A. 2005 Sep 6;102(36):12879-84. Epub 2005 Aug 29.PMID: 16129833 [PubMed - indexed for MEDLINE]Related articlesFree article

[The role of lamins and mutations of LMNA gene in physiological and premature aging]

Sliwińska MA.

Postepy Biochem. 2007;53(1):46-52. Review. Polish. PMID: 17718387 [PubMed - indexed for MEDLINE]Related articles

A homozygous ZMPSTE24 null mutation in combination with a heterozygous mutation in the LMNA gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS.

Denecke J, Brune T, Feldhaus T, Robenek H, Kranz C, Auchus RJ, Agarwal AK, Marquardt T.

Hum Mutat. 2006 Jun;27(6):524-31.PMID: 16671095 [PubMed - indexed for MEDLINE]Related articles

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

Candelario J, Sudhakar S, Navarro S, Reddy S, Comai L.

Aging Cell. 2008 Jun;7(3):355-67. Epub 2008 Mar 24.PMID: 18363904 [PubMed - indexed for MEDLINE]Related articlesFree article

Progeria of stem cells: stem cell exhaustion in Hutchinson-Gilford progeria syndrome.

Halaschek-Wiener J, Brooks-Wilson A.

J Gerontol A Biol Sci Med Sci. 2007 Jan;62(1):3-8. Review.PMID: 17301031 [PubMed - indexed for MEDLINE]Related articles

Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin.

Wang Y, Panteleyev AA, Owens DM, Djabali K, Stewart CL, Worman HJ.

Hum Mol Genet. 2008 Aug 1;17(15):2357-69. Epub 2008 Apr 28.PMID: 18442998 [PubMed - indexed for MEDLINE]Related articlesFree article

Hutchinson-Gilford progeria syndrome: review of the phenotype.

Hennekam RC.

Am J Med Genet A. 2006 Dec 1;140(23):2603-24. Review.PMID: 16838330 [PubMed - indexed for MEDLINE]Related articles

LMNA is mutated in Hutchinson-Gilford progeria (MIM 176670) but not in Wiedemann-Rautenstrauch progeroid syndrome (MIM 264090).

Cao H, Hegele RA.

J Hum Genet. 2003;48(5):271-4. Epub 2003 Apr 3.PMID: 12768443 [PubMed - indexed for MEDLINE]Related articles

The truncated prelamin A in Hutchinson-Gilford progeria syndrome alters segregation of A-type and B-type lamin homopolymers.

Delbarre E, Tramier M, Coppey-Moisan M, Gaillard C, Courvalin JC, Buendia B.

Hum Mol Genet. 2006 Apr 1;15(7):1113-22. Epub 2006 Feb 15.PMID: 16481358 [PubMed - indexed for MEDLINE]Related articlesFree article

Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress.

Paradisi M, McClintock D, Boguslavsky RL, Pedicelli C, Worman HJ, Djabali K.

BMC Cell Biol. 2005 Jun 27;6:27.PMID: 15982412 [PubMed - indexed for MEDLINE]Related articlesFree article

Increased expression of the Hutchinson-Gilford progeria syndrome truncated lamin A transcript during cell aging.

Rodriguez S, Coppedè F, Sagelius H, Eriksson M.

Eur J Hum Genet. 2009 Jul;17(7):928-37. Epub 2009 Jan 28.PMID: 19172989 [PubMed - indexed for MEDLINE]Related articles

Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome.

Varga R, Eriksson M, Erdos MR, Olive M, Harten I, Kolodgie F, Capell BC, Cheng J, Faddah D, Perkins S, Avallone H, San H, Qu X, Ganesh S, Gordon LB, Virmani R, Wight TN, Nabel EG, Collins FS.

Proc Natl Acad Sci U S A. 2006 Feb 28;103(9):3250-5. Epub 2006 Feb 21.PMID: 16492728 [PubMed - indexed for MEDLINE]Related articlesFree article