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Dysregulated interactions between lamin A and SUN1 induce abnormalities in the nuclear envelope and endoplasmic reticulum in progeric laminopathies.

Chen ZJ, Wang WP, Chen YC, Wang JY, Lin WH, Tai LA, Liou GG, Yang CS, Chi YH.

J Cell Sci. 2014 Apr 15;127(Pt 8):1792-804. doi: 10.1242/jcs.139683. Epub 2014 Feb 12.


Reversal of laminopathies: the curious case of SUN1.

Chi YH, Chen CY, Jeang KT.

Nucleus. 2012 Sep-Oct;3(5):418-21. doi: 10.4161/nucl.21714. Epub 2012 Aug 16.


Nuclear localization signal deletion mutants of lamin A and progerin reveal insights into lamin A processing and emerin targeting.

Wu D, Flannery AR, Cai H, Ko E, Cao K.

Nucleus. 2014 Jan-Feb;5(1):66-74. doi: 10.4161/nucl.28068. Epub 2014 Feb 4.


Accumulation of the inner nuclear envelope protein Sun1 is pathogenic in progeric and dystrophic laminopathies.

Chen CY, Chi YH, Mutalif RA, Starost MF, Myers TG, Anderson SA, Stewart CL, Jeang KT.

Cell. 2012 Apr 27;149(3):565-77. doi: 10.1016/j.cell.2012.01.059.


Mammalian SUN protein interaction networks at the inner nuclear membrane and their role in laminopathy disease processes.

Haque F, Mazzeo D, Patel JT, Smallwood DT, Ellis JA, Shanahan CM, Shackleton S.

J Biol Chem. 2010 Jan 29;285(5):3487-98. doi: 10.1074/jbc.M109.071910. Epub 2009 Nov 21.


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.


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.


A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells.

Cao K, Capell BC, Erdos MR, Djabali K, Collins FS.

Proc Natl Acad Sci U S A. 2007 Mar 20;104(12):4949-54. Epub 2007 Mar 14.


Lamin A, farnesylation and aging.

Reddy S, Comai L.

Exp Cell Res. 2012 Jan 1;318(1):1-7. doi: 10.1016/j.yexcr.2011.08.009. Epub 2011 Aug 16. Review.


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.


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.


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.


Hutchinson-Gilford progeria syndrome through the lens of transcription.

Prokocimer M, Barkan R, Gruenbaum Y.

Aging Cell. 2013 Aug;12(4):533-43. doi: 10.1111/acel.12070. Epub 2013 Apr 19. Review.


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.


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.


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.


Epigenetic involvement in Hutchinson-Gilford progeria syndrome: a mini-review.

Arancio W, Pizzolanti G, Genovese SI, Pitrone M, Giordano C.

Gerontology. 2014;60(3):197-203. doi: 10.1159/000357206. Epub 2014 Feb 28. Review.


Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome.

Liu GH, Barkho BZ, Ruiz S, Diep D, Qu J, Yang SL, Panopoulos AD, Suzuki K, Kurian L, Walsh C, Thompson J, Boue S, Fung HL, Sancho-Martinez I, Zhang K, Yates J 3rd, Izpisua Belmonte JC.

Nature. 2011 Apr 14;472(7342):221-5. doi: 10.1038/nature09879. Epub 2011 Feb 23.

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