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Items: 1 to 20 of 209

1.

Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition.

Marji J, O'Donoghue SI, McClintock D, Satagopam VP, Schneider R, Ratner D, Worman HJ, Gordon LB, Djabali K.

PLoS One. 2010 Jun 15;5(6):e11132. doi: 10.1371/journal.pone.0011132.

3.

Identification of mitochondrial dysfunction in Hutchinson-Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture.

Rivera-Torres J, Acín-Perez R, Cabezas-Sánchez P, Osorio FG, Gonzalez-Gómez C, Megias D, Cámara C, López-Otín C, Enríquez JA, Luque-García JL, Andrés V.

J Proteomics. 2013 Oct 8;91:466-77. doi: 10.1016/j.jprot.2013.08.008. Epub 2013 Aug 20.

PMID:
23969228
4.

Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.

McClintock D, Gordon LB, Djabali K.

Proc Natl Acad Sci U S A. 2006 Feb 14;103(7):2154-9. Epub 2006 Feb 6.

5.

Farnesyltransferase inhibitor treatment restores chromosome territory positions and active chromosome dynamics in Hutchinson-Gilford progeria syndrome cells.

Mehta IS, Eskiw CH, Arican HD, Kill IR, Bridger JM.

Genome Biol. 2011 Aug 12;12(8):R74. doi: 10.1186/gb-2011-12-8-r74.

6.

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.

7.

Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts.

Gabriel D, Roedl D, Gordon LB, Djabali K.

Aging Cell. 2015 Feb;14(1):78-91. doi: 10.1111/acel.12300. Epub 2014 Dec 16.

8.

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.

9.

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin.

Yang SH, Chang SY, Ren S, Wang Y, Andres DA, Spielmann HP, Fong LG, Young SG.

Hum Mol Genet. 2011 Feb 1;20(3):436-44. doi: 10.1093/hmg/ddq490. Epub 2010 Nov 18.

11.

A-type lamins and Hutchinson-Gilford progeria syndrome: pathogenesis and therapy.

Gonzalez JM, Pla D, Perez-Sala D, Andres V.

Front Biosci (Schol Ed). 2011 Jun 1;3:1133-46. Review.

PMID:
21622261
12.

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.

Yang SH, Bergo MO, Toth JI, Qiao X, Hu Y, Sandoval S, Meta M, Bendale P, Gelb MH, Young SG, Fong LG.

Proc Natl Acad Sci U S A. 2005 Jul 19;102(29):10291-6. Epub 2005 Jul 12.

13.

LMNA-associated cardiocutaneous progeria: an inherited autosomal dominant premature aging syndrome with late onset.

Kane MS, Lindsay ME, Judge DP, Barrowman J, Ap Rhys C, Simonson L, Dietz HC, Michaelis S.

Am J Med Genet A. 2013 Jul;161A(7):1599-611. doi: 10.1002/ajmg.a.35971. Epub 2013 May 10.

14.
15.

Blocking farnesylation of the prelamin A variant in Hutchinson-Gilford progeria syndrome alters the distribution of A-type lamins.

Wang Y, Ostlund C, Choi JC, Swayne TC, Gundersen GG, Worman HJ.

Nucleus. 2012 Sep-Oct;3(5):452-62. doi: 10.4161/nucl.21675. Epub 2012 Aug 16.

16.

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.

17.

Discordant gene expression signatures and related phenotypic differences in lamin A- and A/C-related Hutchinson-Gilford progeria syndrome (HGPS).

Plasilova M, Chattopadhyay C, Ghosh A, Wenzel F, Demougin P, Noppen C, Schaub N, Szinnai G, Terracciano L, Heinimann K.

PLoS One. 2011;6(6):e21433. doi: 10.1371/journal.pone.0021433. Epub 2011 Jun 27.

18.

Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome.

Mallampalli MP, Huyer G, Bendale P, Gelb MH, Michaelis S.

Proc Natl Acad Sci U S A. 2005 Oct 4;102(40):14416-21. Epub 2005 Sep 26.

19.

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. doi: 10.1093/hmg/ddn136. Epub 2008 Apr 28.

20.

Hutchinson-Gilford progeria syndrome.

Pollex RL, Hegele RA.

Clin Genet. 2004 Nov;66(5):375-81. Review.

PMID:
15479179
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