{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T12:13:40Z","timestamp":1781180020813,"version":"3.54.1"},"reference-count":58,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2016,9,20]],"date-time":"2016-09-20T00:00:00Z","timestamp":1474329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2016,9,20]],"date-time":"2016-09-20T00:00:00Z","timestamp":1474329600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100002920","name":"Research Grants Council, University Grants Committee, Hong Kong","doi-asserted-by":"publisher","award":["T12-705\/11"],"award-info":[{"award-number":["T12-705\/11"]}],"id":[{"id":"10.13039\/501100002920","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Strategic Priority Research Program of the Chinese Academy of Sciences","award":["XDA01020106"],"award-info":[{"award-number":["XDA01020106"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Stem Cell Res Ther"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Laminopathy is a disease closely related to deficiency of the nuclear matrix protein lamin A\/C or failure in prelamin A processing, and leads to accumulation of the misfold protein causing progeria. The resultant disrupted lamin function is highly associated with abnormal nuclear architecture, cell senescence, apoptosis, and unstable genome integrity. To date, the effects of loss in nuclear integrity on the susceptible organ, striated muscle, have been commonly associated with muscular dystrophy, dilated cardiac myopathy (DCM), and conduction defeats, but have not been studied intensively. In this review, we aim to summarize recent breakthroughs in an in vivo laminopathy model and in vitro study using patient-specific human induced pluripotent stem cells (iPSCs) that reproduce the pathophysiological phenotype for further drug screening. We describe several in-vivo transgenic mouse models to elucidate the effects of <jats:italic>Lmna<\/jats:italic> H222P, N195K mutations, and <jats:italic>LMNA<\/jats:italic> knockout on cardiac function, in terms of hemodynamic and electrical signal propagation; certain strategies targeted on stress-related MAPK are mentioned. We will also discuss human iPSC cardiomyocytes serving as a platform to reveal the underlying mechanisms, such as the altered mechanical sensation in electrical coupling of the heart conduction system and ion channel alternation in relation to altered nuclear architecture, and furthermore to enable screening of drugs that can attenuate this cardiac premature aging phenotype by inhibition of prelamin misfolding and oxidative stress, and also enhancement of autophagy protein clearance and cardiac-protective microRNA.<\/jats:p>","DOI":"10.1186\/s13287-016-0401-5","type":"journal-article","created":{"date-parts":[[2016,9,20]],"date-time":"2016-09-20T02:52:45Z","timestamp":1474339965000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Recent advances in animal and human pluripotent stem cell modeling of cardiac laminopathy"],"prefix":"10.1186","volume":"7","author":[{"given":"Yee-Ki","family":"Lee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin-Ru","family":"Ran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yee-Man","family":"Lau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kwong-Man","family":"Ng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wing-Hon Kevin","family":"Lai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chung-Wah","family":"Siu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hung-Fat","family":"Tse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2016,9,20]]},"reference":[{"issue":"22","key":"401_CR1","doi-asserted-by":"crossref","first-page":"16321","DOI":"10.1016\/S0021-9258(19)85424-8","volume":"268","author":"F Lin","year":"1993","unstructured":"Lin F, Worman HJ. Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C. J Biol Chem. 1993;268(22):16321\u20136.","journal-title":"J Biol Chem"},{"issue":"12","key":"401_CR2","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1038\/nrg1906","volume":"7","author":"BC Capell","year":"2006","unstructured":"Capell BC, Collins FS. Human laminopathies: nuclei gone genetically awry. Nat Rev Genet. 2006;7(12):940\u201352.","journal-title":"Nat Rev Genet"},{"issue":"4","key":"401_CR3","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1111\/j.1399-0004.2006.00677.x","volume":"70","author":"J Rankin","year":"2006","unstructured":"Rankin J, Ellard S. The laminopathies: a clinical review. Clin Genet. 2006;70(4):261\u201374.","journal-title":"Clin Genet"},{"issue":"7","key":"401_CR4","doi-asserted-by":"publisher","first-page":"1825","DOI":"10.1172\/JCI37679","volume":"119","author":"HJ Worman","year":"2009","unstructured":"Worman HJ, et al. Laminopathies and the long strange trip from basic cell biology to therapy. J Clin Invest. 2009;119(7):1825\u201336.","journal-title":"J Clin Invest"},{"issue":"4","key":"401_CR5","doi-asserted-by":"publisher","first-page":"331","DOI":"10.5603\/CJ.a2014.0037","volume":"21","author":"F Tesson","year":"2014","unstructured":"Tesson F, et al. Lamin A\/C mutations in dilated cardiomyopathy. Cardiol J. 2014;21(4):331\u201342.","journal-title":"Cardiol J"},{"issue":"11","key":"401_CR6","doi-asserted-by":"publisher","first-page":"803","DOI":"10.18632\/aging.100503","volume":"4","author":"CW Siu","year":"2012","unstructured":"Siu CW, et al. Modeling of lamin A\/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells. Aging (Albany NY). 2012;4(11):803\u201322.","journal-title":"Aging (Albany NY)"},{"issue":"23","key":"401_CR7","doi-asserted-by":"publisher","first-page":"1715","DOI":"10.1056\/NEJM199912023412302","volume":"341","author":"D Fatkin","year":"1999","unstructured":"Fatkin D, et al. Missense mutations in the rod domain of the lamin A\/C gene as causes of dilated cardiomyopathy and conduction-system disease. N Engl J Med. 1999;341(23):1715\u201324.","journal-title":"N Engl J Med"},{"issue":"5","key":"401_CR8","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1016\/S0735-1097(02)02954-6","volume":"41","author":"MR Taylor","year":"2003","unstructured":"Taylor MR, et al. Natural history of dilated cardiomyopathy due to lamin A\/C gene mutations. J Am Coll Cardiol. 2003;41(5):771\u201380.","journal-title":"J Am Coll Cardiol"},{"issue":"5","key":"401_CR9","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1016\/j.hrthm.2009.01.037","volume":"6","author":"H Pan","year":"2009","unstructured":"Pan H, et al. A novel mutation in LAMIN A\/C is associated with isolated early-onset atrial fibrillation and progressive atrioventricular block followed by cardiomyopathy and sudden cardiac death. Heart Rhythm. 2009;6(5):707\u201310.","journal-title":"Heart Rhythm"},{"issue":"2","key":"401_CR10","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.hfc.2009.11.003","volume":"6","author":"D Fatkin","year":"2010","unstructured":"Fatkin D, Otway R, Richmond Z. Genetics of dilated cardiomyopathy. Heart Fail Clin. 2010;6(2):129\u201340.","journal-title":"Heart Fail Clin"},{"key":"401_CR11","doi-asserted-by":"publisher","unstructured":"Dellefave L, McNally EM. The genetics of dilated cardiomyopathy. Curr Opin Cardiol. 2010;25(3):198\u2013204. doi:10.1097\/HCO.0b013e328337ba52.","DOI":"10.1097\/HCO.0b013e328337ba52"},{"issue":"15","key":"401_CR12","doi-asserted-by":"publisher","first-page":"1250","DOI":"10.1016\/j.jacc.2008.06.044","volume":"52","author":"M Pasotti","year":"2008","unstructured":"Pasotti M, et al. Long-term outcome and risk stratification in dilated cardiolaminopathies. J Am Coll Cardiol. 2008;52(15):1250\u201360.","journal-title":"J Am Coll Cardiol"},{"issue":"2","key":"401_CR13","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1056\/NEJMc052632","volume":"354","author":"C Meune","year":"2006","unstructured":"Meune C, et al. Primary prevention of sudden death in patients with lamin A\/C gene mutations. N Engl J Med. 2006;354(2):209\u201310.","journal-title":"N Engl J Med"},{"issue":"11 Pt 1","key":"401_CR14","doi-asserted-by":"publisher","first-page":"1661","DOI":"10.1046\/j.1460-9592.2000.01661.x","volume":"23","author":"HM Becane","year":"2000","unstructured":"Becane HM, et al. High incidence of sudden death with conduction system and myocardial disease due to lamins A and C gene mutation. Pacing Clin Electrophysiol. 2000;23(11 Pt 1):1661\u20136.","journal-title":"Pacing Clin Electrophysiol"},{"key":"401_CR15","doi-asserted-by":"crossref","unstructured":"P\u00e9rez-Serra A, Toro R, Campuzano O, Sarquella-Brugada G, Berne P, Iglesias A, et al. A novel mutation in lamin a\/c causing familial dilated cardiomyopathy associated with sudden cardiac death. J Card Fail. 2014.","DOI":"10.1016\/j.cardfail.2014.12.003"},{"issue":"6","key":"401_CR16","doi-asserted-by":"publisher","first-page":"1081","DOI":"10.1067\/mhj.2002.126737","volume":"144","author":"RE Hershberger","year":"2002","unstructured":"Hershberger RE, et al. A novel lamin A\/C mutation in a family with dilated cardiomyopathy, prominent conduction system disease, and need for permanent pacemaker implantation. Am Heart J. 2002;144(6):1081\u20136.","journal-title":"Am Heart J"},{"issue":"1","key":"401_CR17","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1007\/s00109-004-0589-1","volume":"83","author":"JH van Berlo","year":"2005","unstructured":"van Berlo JH, et al. Meta-analysis of clinical characteristics of 299 carriers of LMNA gene mutations: do lamin A\/C mutations portend a high risk of sudden death? J Mol Med (Berl). 2005;83(1):79\u201383.","journal-title":"J Mol Med (Berl)"},{"issue":"4","key":"401_CR18","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1016\/j.ahj.2014.12.021","volume":"169","author":"P Andre","year":"2015","unstructured":"Andre P, et al. Metabolic and cardiac phenotype characterization in 37 atypical Dunnigan patients with nonfarnesylated mutated prelamin A. Am Heart J. 2015;169(4):587\u201393.","journal-title":"Am Heart J"},{"issue":"3","key":"401_CR19","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1172\/JCI200419448","volume":"113","author":"V Nikolova","year":"2004","unstructured":"Nikolova V, et al. Defects in nuclear structure and function promote dilated cardiomyopathy in lamin A\/C-deficient mice. J Clin Invest. 2004;113(3):357\u201369.","journal-title":"J Clin Invest"},{"issue":"5","key":"401_CR20","doi-asserted-by":"publisher","first-page":"1282","DOI":"10.1172\/JCI29042","volume":"117","author":"A Muchir","year":"2007","unstructured":"Muchir A, et al. Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy. J Clin Invest. 2007;117(5):1282\u201393.","journal-title":"J Clin Invest"},{"issue":"2","key":"401_CR21","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1016\/j.yjmcc.2007.11.008","volume":"44","author":"CM Wolf","year":"2008","unstructured":"Wolf CM, et al. Lamin A\/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease. J Mol Cell Cardiol. 2008;44(2):293\u2013303.","journal-title":"J Mol Cell Cardiol"},{"issue":"6937","key":"401_CR22","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1038\/nature01631","volume":"423","author":"LC Mounkes","year":"2003","unstructured":"Mounkes LC, et al. A progeroid syndrome in mice is caused by defects in A-type lamins. Nature. 2003;423(6937):298\u2013301.","journal-title":"Nature"},{"issue":"8","key":"401_CR23","doi-asserted-by":"publisher","first-page":"1051","DOI":"10.1038\/ejhg.2014.239","volume":"23","author":"F Barthelemy","year":"2015","unstructured":"Barthelemy F, et al. Truncated prelamin A expression in HGPS-like patients: a transcriptional study. Eur J Hum Genet. 2015;23(8):1051\u201361.","journal-title":"Eur J Hum Genet"},{"issue":"1","key":"401_CR24","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1093\/hmg\/ddi017","volume":"14","author":"T Arimura","year":"2005","unstructured":"Arimura T, et al. Mouse model carrying H222P-Lmna mutation develops muscular dystrophy and dilated cardiomyopathy similar to human striated muscle laminopathies. Hum Mol Genet. 2005;14(1):155\u201369.","journal-title":"Hum Mol Genet"},{"issue":"15","key":"401_CR25","doi-asserted-by":"publisher","first-page":"2167","DOI":"10.1093\/hmg\/ddi221","volume":"14","author":"LC Mounkes","year":"2005","unstructured":"Mounkes LC, et al. Expression of an LMNA-N195K variant of A-type lamins results in cardiac conduction defects and death in mice. Hum Mol Genet. 2005;14(15):2167\u201380.","journal-title":"Hum Mol Genet"},{"issue":"5","key":"401_CR26","doi-asserted-by":"publisher","first-page":"913","DOI":"10.1083\/jcb.147.5.913","volume":"147","author":"T Sullivan","year":"1999","unstructured":"Sullivan T, et al. Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy. J Cell Biol. 1999;147(5):913\u201320.","journal-title":"J Cell Biol"},{"issue":"2","key":"401_CR27","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1016\/j.yexcr.2003.07.002","volume":"291","author":"A Muchir","year":"2003","unstructured":"Muchir A, et al. Nuclear envelope alterations in fibroblasts from LGMD1B patients carrying nonsense Y259X heterozygous or homozygous mutation in lamin A\/C gene. Exp Cell Res. 2003;291(2):352\u201362.","journal-title":"Exp Cell Res"},{"issue":"3","key":"401_CR28","doi-asserted-by":"publisher","first-page":"195","DOI":"10.4161\/nucl.2.3.15731","volume":"2","author":"N Kubben","year":"2011","unstructured":"Kubben N, et al. Post-natal myogenic and adipogenic developmental: defects and metabolic impairment upon loss of A-type lamins. Nucleus. 2011;2(3):195\u2013207.","journal-title":"Nucleus"},{"issue":"1","key":"401_CR29","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1016\/j.bbrc.2013.08.082","volume":"440","author":"Y Kim","year":"2013","unstructured":"Kim Y, Zheng Y. Generation and characterization of a conditional deletion allele for Lmna in mice. Biochem Biophys Res Commun. 2013;440(1):8\u201313.","journal-title":"Biochem Biophys Res Commun"},{"issue":"3","key":"401_CR30","doi-asserted-by":"publisher","first-page":"e75","DOI":"10.1016\/j.ijcard.2009.04.036","volume":"137","author":"P Ambrosi","year":"2009","unstructured":"Ambrosi P, et al. Heart transplantation in 7 patients from a single family with limb-girdle muscular dystrophy caused by lamin A\/C mutation. Int J Cardiol. 2009;137(3):e75\u20136.","journal-title":"Int J Cardiol"},{"issue":"10","key":"401_CR31","doi-asserted-by":"publisher","first-page":"1492","DOI":"10.1016\/j.hrthm.2013.06.020","volume":"10","author":"F Anselme","year":"2013","unstructured":"Anselme F, et al. Implantable cardioverter-defibrillators in lamin A\/C mutation carriers with cardiac conduction disorders. Heart Rhythm. 2013;10(10):1492\u20138.","journal-title":"Heart Rhythm"},{"issue":"1","key":"401_CR32","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1093\/hmg\/ddt387","volume":"23","author":"W Wu","year":"2014","unstructured":"Wu W, et al. Depletion of extracellular signal-regulated kinase 1 in mice with cardiomyopathy caused by lamin A\/C gene mutation partially prevents pathology before isoenzyme activation. Hum Mol Genet. 2014;23(1):1\u201311.","journal-title":"Hum Mol Genet"},{"issue":"7\u20138","key":"401_CR33","doi-asserted-by":"publisher","first-page":"632","DOI":"10.1016\/j.bbadis.2010.04.001","volume":"1802","author":"W Wu","year":"2010","unstructured":"Wu W, et al. Pharmacological inhibition of c-Jun N-terminal kinase signaling prevents cardiomyopathy caused by mutation in LMNA gene. Biochim Biophys Acta. 2010;1802(7\u20138):632\u20138.","journal-title":"Biochim Biophys Acta"},{"issue":"1","key":"401_CR34","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1161\/CIRCULATIONAHA.110.970673","volume":"123","author":"W Wu","year":"2011","unstructured":"Wu W, et al. Mitogen-activated protein kinase inhibitors improve heart function and prevent fibrosis in cardiomyopathy caused by mutation in lamin A\/C gene. Circulation. 2011;123(1):53\u201361.","journal-title":"Circulation"},{"issue":"19","key":"401_CR35","doi-asserted-by":"publisher","first-page":"4325","DOI":"10.1093\/hmg\/dds265","volume":"21","author":"A Muchir","year":"2012","unstructured":"Muchir A, et al. Abnormal p38alpha mitogen-activated protein kinase signaling in dilated cardiomyopathy caused by lamin A\/C gene mutation. Hum Mol Genet. 2012;21(19):4325\u201333.","journal-title":"Hum Mol Genet"},{"issue":"144","key":"401_CR36","doi-asserted-by":"publisher","first-page":"144ra102","DOI":"10.1126\/scitranslmed.3003875","volume":"4","author":"JC Choi","year":"2012","unstructured":"Choi JC, et al. Temsirolimus activates autophagy and ameliorates cardiomyopathy caused by lamin A\/C gene mutation. Sci Transl Med. 2012;4(144):144ra102.","journal-title":"Sci Transl Med"},{"issue":"5","key":"401_CR37","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1016\/j.cell.2007.11.019","volume":"131","author":"K Takahashi","year":"2007","unstructured":"Takahashi K, et al. Induction of pluripotent stem cells from adult human fibroblasts by defined factors. Cell. 2007;131(5):861\u201372.","journal-title":"Cell"},{"issue":"5858","key":"401_CR38","doi-asserted-by":"publisher","first-page":"1917","DOI":"10.1126\/science.1151526","volume":"318","author":"J Yu","year":"2007","unstructured":"Yu J, et al. Induced pluripotent stem cell lines derived from human somatic cells. Science. 2007;318(5858):1917\u201320.","journal-title":"Science"},{"issue":"15","key":"401_CR39","doi-asserted-by":"publisher","first-page":"1397","DOI":"10.1056\/NEJMoa0908679","volume":"363","author":"A Moretti","year":"2010","unstructured":"Moretti A, et al. Patient-specific induced pluripotent stem-cell models for long-QT syndrome. N Engl J Med. 2010;363(15):1397\u2013409.","journal-title":"N Engl J Med"},{"issue":"7337","key":"401_CR40","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1038\/nature09747","volume":"471","author":"I Itzhaki","year":"2011","unstructured":"Itzhaki I, et al. Modelling the long QT syndrome with induced pluripotent stem cells. Nature. 2011;471(7337):225\u20139.","journal-title":"Nature"},{"issue":"7337","key":"401_CR41","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1038\/nature09855","volume":"471","author":"M Yazawa","year":"2011","unstructured":"Yazawa M, et al. Using induced pluripotent stem cells to investigate cardiac phenotypes in Timothy syndrome. Nature. 2011;471(7337):230\u20134.","journal-title":"Nature"},{"issue":"7342","key":"401_CR42","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1038\/nature09879","volume":"472","author":"GH Liu","year":"2011","unstructured":"Liu GH, et al. Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome. Nature. 2011;472(7342):221\u20135.","journal-title":"Nature"},{"issue":"1","key":"401_CR43","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.stem.2010.12.002","volume":"8","author":"J Zhang","year":"2011","unstructured":"Zhang J, et al. A human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defects. Cell Stem Cell. 2011;8(1):31\u201345.","journal-title":"Cell Stem Cell"},{"issue":"4","key":"401_CR44","doi-asserted-by":"publisher","first-page":"380","DOI":"10.18632\/aging.100277","volume":"3","author":"JC Ho","year":"2011","unstructured":"Ho JC, et al. Generation of induced pluripotent stem cell lines from 3 distinct laminopathies bearing heterogeneous mutations in lamin A\/C. Aging (Albany NY). 2011;3(4):380\u201390.","journal-title":"Aging (Albany NY)"},{"issue":"4","key":"401_CR45","doi-asserted-by":"publisher","first-page":"510","DOI":"10.5966\/sctm.2013-0168","volume":"3","author":"S Blondel","year":"2014","unstructured":"Blondel S, et al. Induced pluripotent stem cells reveal functional differences between drugs currently investigated in patients with hutchinson-gilford progeria syndrome. Stem Cells Transl Med. 2014;3(4):510\u20139.","journal-title":"Stem Cells Transl Med"},{"issue":"17","key":"401_CR46","doi-asserted-by":"publisher","first-page":"7686","DOI":"10.1073\/pnas.92.17.7686","volume":"92","author":"DT Dudley","year":"1995","unstructured":"Dudley DT, et al. A synthetic inhibitor of the mitogen-activated protein kinase cascade. Proc Natl Acad Sci U S A. 1995;92(17):7686\u20139.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"401_CR47","doi-asserted-by":"publisher","first-page":"688","DOI":"10.1016\/j.stem.2011.04.019","volume":"8","author":"GH Liu","year":"2011","unstructured":"Liu GH, et al. Targeted gene correction of laminopathy-associated LMNA mutations in patient-specific iPSCs. Cell Stem Cell. 2011;8(6):688\u201394.","journal-title":"Cell Stem Cell"},{"issue":"8","key":"401_CR48","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1038\/ncb3207","volume":"17","author":"C Soria-Valles","year":"2015","unstructured":"Soria-Valles C, et al. NF-kappaB activation impairs somatic cell reprogramming in ageing. Nat Cell Biol. 2015;17(8):1004\u201313.","journal-title":"Nat Cell Biol"},{"issue":"6","key":"401_CR49","doi-asserted-by":"publisher","first-page":"1017","DOI":"10.1182\/blood-2013-04-497644","volume":"122","author":"SR Daigle","year":"2013","unstructured":"Daigle SR, et al. Potent inhibition of DOT1L as treatment of MLL-fusion leukemia. Blood. 2013;122(6):1017\u201325.","journal-title":"Blood"},{"issue":"1","key":"401_CR50","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.celrep.2012.05.015","volume":"2","author":"X Nissan","year":"2012","unstructured":"Nissan X, et al. Unique preservation of neural cells in Hutchinson-Gilford progeria syndrome is due to the expression of the neural-specific miR-9 microRNA. Cell Rep. 2012;2(1):1\u20139.","journal-title":"Cell Rep"},{"issue":"10","key":"401_CR51","doi-asserted-by":"publisher","first-page":"R115","DOI":"10.1186\/gb-2013-14-10-r115","volume":"14","author":"S Horvath","year":"2013","unstructured":"Horvath S. DNA methylation age of human tissues and cell types. Genome Biol. 2013;14(10):R115.","journal-title":"Genome Biol"},{"issue":"3","key":"401_CR52","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1093\/cvr\/cvt106","volume":"99","author":"T Arimura","year":"2013","unstructured":"Arimura T, et al. Nuclear accumulation of androgen receptor in gender difference of dilated cardiomyopathy due to lamin A\/C mutations. Cardiovasc Res. 2013;99(3):382\u201394.","journal-title":"Cardiovasc Res"},{"issue":"1","key":"401_CR53","doi-asserted-by":"publisher","first-page":"110","DOI":"10.4161\/auto.22403","volume":"9","author":"JC Choi","year":"2013","unstructured":"Choi JC, Worman HJ. Reactivation of autophagy ameliorates LMNA cardiomyopathy. Autophagy. 2013;9(1):110\u20131.","journal-title":"Autophagy"},{"issue":"3","key":"401_CR54","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1054\/jcaf.2001.26339","volume":"7","author":"PM Jakobs","year":"2001","unstructured":"Jakobs PM, et al. Novel lamin A\/C mutations in two families with dilated cardiomyopathy and conduction system disease. J Card Fail. 2001;7(3):249\u201356.","journal-title":"J Card Fail"},{"issue":"4","key":"401_CR55","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1620\/tjem.218.309","volume":"218","author":"A Saga","year":"2009","unstructured":"Saga A, et al. Lamin A\/C gene mutations in familial cardiomyopathy with advanced atrioventricular block and arrhythmia. Tohoku J Exp Med. 2009;218(4):309\u201316.","journal-title":"Tohoku J Exp Med"},{"issue":"6","key":"401_CR56","doi-asserted-by":"publisher","first-page":"1130","DOI":"10.1016\/j.ahj.2007.07.038","volume":"154","author":"JP van Tintelen","year":"2007","unstructured":"van Tintelen JP, et al. High yield of LMNA mutations in patients with dilated cardiomyopathy and\/or conduction disease referred to cardiogenetics outpatient clinics. Am Heart J. 2007;154(6):1130\u20139.","journal-title":"Am Heart J"},{"issue":"12","key":"401_CR57","doi-asserted-by":"publisher","first-page":"2080","DOI":"10.1038\/nprot.2012.115","volume":"7","author":"T Zhou","year":"2012","unstructured":"Zhou T, et al. Generation of human induced pluripotent stem cells from urine samples. Nat Protoc. 2012;7(12):2080\u20139.","journal-title":"Nat Protoc"},{"issue":"6","key":"401_CR58","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1089\/cell.2010.0051","volume":"12","author":"WH Lai","year":"2010","unstructured":"Lai WH, et al. ROCK inhibition facilitates the generation of human-induced pluripotent stem cells in a defined, feeder-, and serum-free system. Cell Reprogram. 2010;12(6):641\u201353.","journal-title":"Cell Reprogram"}],"container-title":["Stem Cell Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13287-016-0401-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13287-016-0401-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13287-016-0401-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T06:21:19Z","timestamp":1637043679000},"score":1,"resource":{"primary":{"URL":"https:\/\/stemcellres.biomedcentral.com\/articles\/10.1186\/s13287-016-0401-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9,20]]},"references-count":58,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2016,12]]}},"alternative-id":["401"],"URL":"https:\/\/doi.org\/10.1186\/s13287-016-0401-5","relation":{},"ISSN":["1757-6512"],"issn-type":[{"value":"1757-6512","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,20]]},"assertion":[{"value":"20 September 2016","order":1,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"139"}}