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Items: 1 to 50 of 216

1.

Nuclear envelope-localized torsinA-LAP1 complex regulates hepatic VLDL secretion and steatosis.

Shin JY, Hernandez-Ono A, Fedotova T, Östlund C, Lee MJ, Gibeley SB, Liang CC, Dauer WT, Ginsberg HN, Worman HJ.

J Clin Invest. 2019 Aug 13;130. pii: 129769. doi: 10.1172/JCI129769.

2.

Pathogenic mutations in genes encoding nuclear envelope proteins and defective nucleocytoplasmic connections.

Östlund C, Chang W, Gundersen GG, Worman HJ.

Exp Biol Med (Maywood). 2019 Jul 12:1535370219862243. doi: 10.1177/1535370219862243. [Epub ahead of print] No abstract available.

PMID:
31299860
3.

Postnatal Development of Mice with Combined Genetic Depletions of Lamin A/C, Emerin and Lamina-associated Polypeptide 1.

Wang Y, Shin JY, Nakanishi K, Homma S, Kim GJ, Tanji K, Joseph LC, Morrow JP, Stewart CL, Dauer WT, Worman HJ.

Hum Mol Genet. 2019 Apr 22. pii: ddz082. doi: 10.1093/hmg/ddz082. [Epub ahead of print]

PMID:
31009944
4.

Imbalanced nucleocytoskeletal connections create common polarity defects in progeria and physiological aging.

Chang W, Wang Y, Luxton GWG, Östlund C, Worman HJ, Gundersen GG.

Proc Natl Acad Sci U S A. 2019 Feb 26;116(9):3578-3583. doi: 10.1073/pnas.1809683116. Epub 2019 Feb 11.

5.

Next-Generation Sequencing and Mutational Analysis: Implications for Genes Encoding LINC Complex Proteins.

Nagy PL, Worman HJ.

Methods Mol Biol. 2018;1840:321-336. doi: 10.1007/978-1-4939-8691-0_22.

PMID:
30141054
6.

Permanently Farnesylated Prelamin A, Progeria, and Atherosclerosis.

Worman HJ, Michaelis S.

Circulation. 2018 Jul 17;138(3):283-286. doi: 10.1161/CIRCULATIONAHA.118.034480. No abstract available.

7.

Cofilin-1 phosphorylation catalyzed by ERK1/2 alters cardiac actin dynamics in dilated cardiomyopathy caused by lamin A/C gene mutation.

Chatzifrangkeskou M, Yadin D, Marais T, Chardonnet S, Cohen-Tannoudji M, Mougenot N, Schmitt A, Crasto S, Di Pasquale E, Macquart C, Tanguy Y, Jebeniani I, Pucéat M, Morales Rodriguez B, Goldmann WH, Dal Ferro M, Biferi MG, Knaus P, Bonne G, Worman HJ, Muchir A.

Hum Mol Genet. 2018 Sep 1;27(17):3060-3078. doi: 10.1093/hmg/ddy215.

8.

Elevated dual specificity protein phosphatase 4 in cardiomyopathy caused by lamin A/C gene mutation is primarily ERK1/2-dependent and its depletion improves cardiac function and survival.

Choi JC, Wu W, Phillips E, Plevin R, Sera F, Homma S, Worman HJ.

Hum Mol Genet. 2018 Jul 1;27(13):2290-2305. doi: 10.1093/hmg/ddy134.

9.

Cell signaling abnormalities in cardiomyopathy caused by lamin A/C gene mutations.

Worman HJ.

Biochem Soc Trans. 2018 Feb 19;46(1):37-42. doi: 10.1042/BST20170236. Epub 2017 Dec 1. Review.

10.

TorsinA controls TAN line assembly and the retrograde flow of dorsal perinuclear actin cables during rearward nuclear movement.

Saunders CA, Harris NJ, Willey PT, Woolums BM, Wang Y, McQuown AJ, Schoenhofen A, Worman HJ, Dauer WT, Gundersen GG, Luxton GW.

J Cell Biol. 2017 Mar 6;216(3):657-674. doi: 10.1083/jcb.201507113. Epub 2017 Feb 27.

11.

Decreased WNT/β-catenin signalling contributes to the pathogenesis of dilated cardiomyopathy caused by mutations in the lamin a/C gene.

Le Dour C, Macquart C, Sera F, Homma S, Bonne G, Morrow JP, Worman HJ, Muchir A.

Hum Mol Genet. 2017 Jan 15;26(2):333-343. doi: 10.1093/hmg/ddw389.

12.

Macrocyclic MEK1/2 inhibitor with efficacy in a mouse model of cardiomyopathy caused by lamin A/C gene mutation.

Wu W, Chordia MD, Hart BP, Kumarasinghe ES, Ji MK, Bhargava A, Lawlor MW, Shin JY, Sera F, Homma S, Muchir A, Khire UR, Worman HJ.

Bioorg Med Chem. 2017 Feb 1;25(3):1004-1013. doi: 10.1016/j.bmc.2016.12.014. Epub 2016 Dec 9.

13.

Extracellular matrix remodeling and transforming growth factor-β signaling abnormalities induced by lamin A/C variants that cause lipodystrophy.

Le Dour C, Wu W, Béréziat V, Capeau J, Vigouroux C, Worman HJ.

J Lipid Res. 2017 Jan;58(1):151-163. doi: 10.1194/jlr.M071381. Epub 2016 Nov 14.

14.

Lamina-associated polypeptide 1 is dispensable for embryonic myogenesis but required for postnatal skeletal muscle growth.

Shin JY, Méndez-López I, Hong M, Wang Y, Tanji K, Wu W, Shugol L, Krauss RS, Dauer WT, Worman HJ.

Hum Mol Genet. 2017 Jan 1;26(1):65-78. doi: 10.1093/hmg/ddw368.

15.

ERK1/2 directly acts on CTGF/CCN2 expression to mediate myocardial fibrosis in cardiomyopathy caused by mutations in the lamin A/C gene.

Chatzifrangkeskou M, Le Dour C, Wu W, Morrow JP, Joseph LC, Beuvin M, Sera F, Homma S, Vignier N, Mougenot N, Bonne G, Lipson KE, Worman HJ, Muchir A.

Hum Mol Genet. 2016 Jun 1;25(11):2220-2233. Epub 2016 Apr 30.

16.

A mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder.

Wang Y, Lichter-Konecki U, Anyane-Yeboa K, Shaw JE, Lu JT, Östlund C, Shin JY, Clark LN, Gundersen GG, Nagy PL, Worman HJ.

J Cell Sci. 2016 May 15;129(10):1975-80. doi: 10.1242/jcs.187302. Epub 2016 Mar 31.

17.

Genome-wide association study in mice identifies loci affecting liver-related phenotypes including Sel1l influencing serum bile acids.

Wu W, Patel A, Kyöstilä K, Lohi H, Mladkova N, Kiryluk K, Sun X, Lefkowitch JH, Worman HJ, Gharavi AG.

Hepatology. 2016 Jun;63(6):1943-56. doi: 10.1002/hep.28495. Epub 2016 Mar 24.

PMID:
26857093
18.

Targeting Mitogen-Activated Protein Kinase Signaling in Mouse Models of Cardiomyopathy Caused by Lamin A/C Gene Mutations.

Muchir A, Worman HJ.

Methods Enzymol. 2016;568:557-80. doi: 10.1016/bs.mie.2015.07.028. Epub 2015 Oct 24.

19.

Purification and Structural Analysis of LEM-Domain Proteins.

Herrada I, Bourgeois B, Samson C, Buendia B, Worman HJ, Zinn-Justin S.

Methods Enzymol. 2016;569:43-61. doi: 10.1016/bs.mie.2015.07.008. Epub 2015 Aug 8.

PMID:
26778552
20.

Muscular Dystrophy Mutations Impair the Nuclear Envelope Emerin Self-assembly Properties.

Herrada I, Samson C, Velours C, Renault L, Östlund C, Chervy P, Puchkov D, Worman HJ, Buendia B, Zinn-Justin S.

ACS Chem Biol. 2015 Dec 18;10(12):2733-42. doi: 10.1021/acschembio.5b00648. Epub 2015 Oct 5.

21.

Nuclear membrane diversity: underlying tissue-specific pathologies in disease?

Worman HJ, Schirmer EC.

Curr Opin Cell Biol. 2015 Jun;34:101-12. doi: 10.1016/j.ceb.2015.06.003. Epub 2015 Jun 24. Review.

22.

Acute liver injury associated with a newer formulation of the herbal weight loss supplement Hydroxycut.

Araujo JL, Worman HJ.

BMJ Case Rep. 2015 May 6;2015. pii: bcr2015210303. doi: 10.1136/bcr-2015-210303.

23.

Linker of nucleoskeleton and cytoskeleton (LINC) complex-mediated actin-dependent nuclear positioning orients centrosomes in migrating myoblasts.

Chang W, Antoku S, Östlund C, Worman HJ, Gundersen GG.

Nucleus. 2015;6(1):77-88. doi: 10.1080/19491034.2015.1004947.

24.

Accessorizing and anchoring the LINC complex for multifunctionality.

Chang W, Worman HJ, Gundersen GG.

J Cell Biol. 2015 Jan 5;208(1):11-22. doi: 10.1083/jcb.201409047. Review.

25.

Neurofilament light polypeptide gene N98S mutation in mice leads to neurofilament network abnormalities and a Charcot-Marie-Tooth Type 2E phenotype.

Adebola AA, Di Castri T, He CZ, Salvatierra LA, Zhao J, Brown K, Lin CS, Worman HJ, Liem RK.

Hum Mol Genet. 2015 Apr 15;24(8):2163-74. doi: 10.1093/hmg/ddu736. Epub 2014 Dec 30.

26.

Cellular stress induces Bax-regulated nuclear bubble budding and rupture followed by nuclear protein release.

Lindenboim L, Sasson T, Worman HJ, Borner C, Stein R.

Nucleus. 2014;5(6):527-41. doi: 10.4161/19491034.2014.970105.

27.

Michael Field, MD (1933-2014).

Chang EB, Guandalini S, Rao MC, Sellin JH, Semrad CE, Worman H.

Gastroenterology. 2014 Dec;147(6):1191-2. doi: 10.1053/j.gastro.2014.10.026. Epub 2014 Oct 25. No abstract available.

PMID:
25449022
28.

Mitogen-activated protein kinase kinase 1/2 inhibition and angiotensin II converting inhibition in mice with cardiomyopathy caused by lamin A/C gene mutation.

Muchir A, Wu W, Sera F, Homma S, Worman HJ.

Biochem Biophys Res Commun. 2014 Oct 3;452(4):958-61. doi: 10.1016/j.bbrc.2014.09.020. Epub 2014 Sep 11.

29.

Muscular dystrophy-associated SUN1 and SUN2 variants disrupt nuclear-cytoskeletal connections and myonuclear organization.

Meinke P, Mattioli E, Haque F, Antoku S, Columbaro M, Straatman KR, Worman HJ, Gundersen GG, Lattanzi G, Wehnert M, Shackleton S.

PLoS Genet. 2014 Sep 11;10(9):e1004605. doi: 10.1371/journal.pgen.1004605. eCollection 2014 Sep.

30.

The nuclear envelope: an intriguing focal point for neurogenetic disease.

Worman HJ, Dauer WT.

Neurotherapeutics. 2014 Oct;11(4):764-72. doi: 10.1007/s13311-014-0296-8. Review.

31.

Depletion of lamina-associated polypeptide 1 from cardiomyocytes causes cardiac dysfunction in mice.

Shin JY, Le Dour C, Sera F, Iwata S, Homma S, Joseph LC, Morrow JP, Dauer WT, Worman HJ.

Nucleus. 2014 May-Jun;5(3):260-459. doi: 10.4161/nucl.29227. Epub 2014 May 23.

32.

Nuclear envelope regulation of signaling cascades.

Choi JC, Worman HJ.

Adv Exp Med Biol. 2014;773:187-206. doi: 10.1007/978-1-4899-8032-8_9. Review.

PMID:
24563349
33.

Lamina-associated polypeptide 1: protein interactions and tissue-selective functions.

Shin JY, Dauer WT, Worman HJ.

Semin Cell Dev Biol. 2014 May;29:164-8. doi: 10.1016/j.semcdb.2014.01.010. Epub 2014 Feb 5. Review.

34.

Emerin organizes actin flow for nuclear movement and centrosome orientation in migrating fibroblasts.

Chang W, Folker ES, Worman HJ, Gundersen GG.

Mol Biol Cell. 2013 Dec;24(24):3869-80. doi: 10.1091/mbc.E13-06-0307. Epub 2013 Oct 23.

35.

Lamina-associated polypeptide-1 interacts with the muscular dystrophy protein emerin and is essential for skeletal muscle maintenance.

Shin JY, Méndez-López I, Wang Y, Hays AP, Tanji K, Lefkowitch JH, Schulze PC, Worman HJ, Dauer WT.

Dev Cell. 2013 Sep 30;26(6):591-603. doi: 10.1016/j.devcel.2013.08.012. Epub 2013 Sep 19.

36.

Depletion of extracellular signal-regulated kinase 1 in mice with cardiomyopathy caused by lamin A/C gene mutation partially prevents pathology before isoenzyme activation.

Wu W, Iwata S, Homma S, Worman HJ, Muchir A.

Hum Mol Genet. 2014 Jan 1;23(1):1-11. doi: 10.1093/hmg/ddt387. Epub 2013 Aug 9.

37.
38.

Inhibition of TGF-β signaling at the nuclear envelope: characterization of interactions between MAN1, Smad2 and Smad3, and PPM1A.

Bourgeois B, Gilquin B, Tellier-Lebègue C, Östlund C, Wu W, Pérez J, El Hage P, Lallemand F, Worman HJ, Zinn-Justin S.

Sci Signal. 2013 Jun 18;6(280):ra49. doi: 10.1126/scisignal.2003411.

39.

Nuclear positioning.

Gundersen GG, Worman HJ.

Cell. 2013 Mar 14;152(6):1376-89. doi: 10.1016/j.cell.2013.02.031. Review.

40.

Nucleocytoplasmic connections and deafness.

Worman HJ, Segil N.

J Clin Invest. 2013 Feb;123(2):553-5. doi: 10.1172/JCI67454. Epub 2013 Jan 25.

41.

Lamin A/C depletion enhances DNA damage-induced stalled replication fork arrest.

Singh M, Hunt CR, Pandita RK, Kumar R, Yang CR, Horikoshi N, Bachoo R, Serag S, Story MD, Shay JW, Powell SN, Gupta A, Jeffery J, Pandita S, Chen BP, Deckbar D, Löbrich M, Yang Q, Khanna KK, Worman HJ, Pandita TK.

Mol Cell Biol. 2013 Mar;33(6):1210-22. doi: 10.1128/MCB.01676-12. Epub 2013 Jan 14. Erratum in: Mol Cell Biol. 2013 Aug;33(16):3390.

42.

Dual specificity phosphatase 4 mediates cardiomyopathy caused by lamin A/C (LMNA) gene mutation.

Choi JC, Wu W, Muchir A, Iwata S, Homma S, Worman HJ.

J Biol Chem. 2012 Nov 23;287(48):40513-24. doi: 10.1074/jbc.M112.404541. Epub 2012 Oct 9.

43.

Reactivation of autophagy ameliorates LMNA cardiomyopathy.

Choi JC, Worman HJ.

Autophagy. 2013 Jan;9(1):110-1. doi: 10.4161/auto.22403. Epub 2012 Oct 8.

44.

Cardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- mice.

Frock RL, Chen SC, Da DF, Frett E, Lau C, Brown C, Pak DN, Wang Y, Muchir A, Worman HJ, Santana LF, Ladiges WC, Rabinovitch PS, Kennedy BK.

PLoS One. 2012;7(8):e42918. doi: 10.1371/journal.pone.0042918. Epub 2012 Aug 15.

45.

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.

46.

Temsirolimus activates autophagy and ameliorates cardiomyopathy caused by lamin A/C gene mutation.

Choi JC, Muchir A, Wu W, Iwata S, Homma S, Morrow JP, Worman HJ.

Sci Transl Med. 2012 Jul 25;4(144):144ra102. doi: 10.1126/scitranslmed.3003875.

47.

Abnormal p38α mitogen-activated protein kinase signaling in dilated cardiomyopathy caused by lamin A/C gene mutation.

Muchir A, Wu W, Choi JC, Iwata S, Morrow J, Homma S, Worman HJ.

Hum Mol Genet. 2012 Oct 1;21(19):4325-33. doi: 10.1093/hmg/dds265. Epub 2012 Jul 5.

48.

Epitope-specific anti-nuclear antibodies are expressed in a mouse model of primary biliary cirrhosis and are cytokine-dependent.

Yang CY, Leung PS, Yang GX, Kenny TP, Zhang W, Coppel R, Norman GL, Ansari AA, Mackay IR, Worman HJ, Gershwin ME.

Clin Exp Immunol. 2012 Jun;168(3):261-7. doi: 10.1111/j.1365-2249.2012.04577.x.

49.

Primary biliary cirrhosis and the nuclear pore complex.

Duarte-Rey C, Bogdanos D, Yang CY, Roberts K, Leung PS, Anaya JM, Worman HJ, Gershwin ME.

Autoimmun Rev. 2012 Oct;11(12):898-902. doi: 10.1016/j.autrev.2012.03.005. Epub 2012 Apr 1. Review.

50.

Inner nuclear membrane proteins: impact on human disease.

Méndez-López I, Worman HJ.

Chromosoma. 2012 Apr;121(2):153-67. doi: 10.1007/s00412-012-0360-2. Epub 2012 Feb 4. Review.

PMID:
22307332

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