{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T03:00:22Z","timestamp":1787022022392,"version":"build-2736575974"},"reference-count":33,"publisher":"Public Library of Science (PLoS)","issue":"5","license":[{"start":{"date-parts":[[2018,5,15]],"date-time":"2018-05-15T00:00:00Z","timestamp":1526342400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","award":["113302001"],"award-info":[{"award-number":["113302001"]}],"id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]},{"name":"NeurOmics","award":["FP7, Agreement 2012-305121"],"award-info":[{"award-number":["FP7, Agreement 2012-305121"]}]},{"DOI":"10.13039\/501100004923","name":"AFM-T\u00e9l\u00e9thon","doi-asserted-by":"publisher","award":["20251"],"award-info":[{"award-number":["20251"]}],"id":[{"id":"10.13039\/501100004923","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0197388","type":"journal-article","created":{"date-parts":[[2018,5,15]],"date-time":"2018-05-15T16:34:29Z","timestamp":1526402069000},"page":"e0197388","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":8,"title":["Natural disease history of the dy2J mouse model of laminin \u03b12 (merosin)-deficient congenital muscular dystrophy"],"prefix":"10.1371","volume":"13","author":[{"given":"S.","family":"Pasteuning-Vuhman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Putker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. L.","family":"Tanganyika-de Winter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. W.","family":"Boertje-van der Meulen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"van Vliet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Overzier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. J.","family":"Plomp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Aartsma-Rus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"van Putten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2018,5,15]]},"reference":[{"issue":"7\u20138","key":"ref1","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1016\/S0960-8966(03)00072-5","article-title":"114th ENMC International Workshop on Congenital Muscular Dystrophy (CMD) 17\u201319 January 2003, Naarden, The Netherlands: (8th Workshop of the International Consortium on CMD; 3rd Workshop of the MYO-CLUSTER project GENRE)","volume":"13","author":"F Muntoni","year":"2003","journal-title":"Neuromuscular disorders: NMD"},{"issue":"2","key":"ref2","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1038\/sj.ejhg.5200743","article-title":"Merosin-deficient congenital muscular dystrophy, autosomal recessive (MDC1A, MIM#156225, LAMA2 gene coding for alpha2 chain of laminin)","volume":"10","author":"V Allamand","year":"2002","journal-title":"European journal of human genetics: EJHG"},{"issue":"3","key":"ref3","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1111\/cge.12366","article-title":"Genotype\/phenotype analysis in Chinese laminin-alpha2 deficient congenital muscular dystrophy patients","volume":"87","author":"H Xiong","year":"2015","journal-title":"Clinical genetics"},{"issue":"4","key":"ref4","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.nmd.2010.02.001","article-title":"Genotype-phenotype correlation in a large population of muscular dystrophy patients with LAMA2 mutations","volume":"20","author":"F Geranmayeh","year":"2010","journal-title":"Neuromuscular disorders: NMD"},{"issue":"1","key":"ref5","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/2044-5040-1-9","article-title":"Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies","volume":"1","author":"KI Gawlik","year":"2011","journal-title":"Skeletal muscle"},{"issue":"1","key":"ref6","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/S0014-5793(97)01007-7","article-title":"Laminin alpha2 chain-null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)-deficient congenital muscular dystrophy","volume":"415","author":"Y Miyagoe","year":"1997","journal-title":"FEBS letters"},{"issue":"3","key":"ref7","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1038\/ng1194-297","article-title":"Murine muscular dystrophy caused by a mutation in the laminin alpha 2 (Lama2) gene","volume":"8","author":"H Xu","year":"1994","journal-title":"Nature genetics"},{"issue":"6","key":"ref8","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1093\/hmg\/4.6.1055","article-title":"Identification of a novel mutant transcript of laminin alpha 2 chain gene responsible for muscular dystrophy and dysmyelination in dy2J mice","volume":"4","author":"Y Sunada","year":"1995","journal-title":"Human molecular genetics"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/bs.ctm.2015.05.002","article-title":"Laminin-alpha2 Chain-Deficient Congenital Muscular Dystrophy: Pathophysiology and Development of Treatment","volume":"76","author":"M Durbeej","year":"2015","journal-title":"Current topics in membranes"},{"issue":"4","key":"ref10","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.nmd.2010.02.004","article-title":"A 3 months mild functional test regime does not affect disease parameters in young mdx mice","volume":"20","author":"M van Putten","year":"2010","journal-title":"Neuromuscular disorders: NMD"},{"issue":"8","key":"ref11","doi-asserted-by":"crossref","first-page":"e0182704","DOI":"10.1371\/journal.pone.0182704","article-title":"Natural disease history of mouse models for limb girdle muscular dystrophy types 2D and 2F","volume":"12","author":"S Pasteuning-Vuhman","year":"2017","journal-title":"PloS one"},{"issue":"85","key":"ref12","doi-asserted-by":"crossref","DOI":"10.3791\/51303","article-title":"Assessing functional performance in the mdx mouse model","author":"A Aartsma-Rus","year":"2014","journal-title":"Journal of visualized experiments: JoVE"},{"issue":"12","key":"ref13","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.1111\/ejn.13249","article-title":"Characterization of neuromuscular synapse function abnormalities in multiple Duchenne muscular dystrophy mouse models","volume":"43","author":"EM van der Pijl","year":"2016","journal-title":"The European journal of neuroscience"},{"issue":"6","key":"ref14","doi-asserted-by":"crossref","first-page":"e45","DOI":"10.1093\/nar\/gkp045","article-title":"Amplification efficiency: linking baseline and bias in the analysis of quantitative PCR data","volume":"37","author":"JM Ruijter","year":"2009","journal-title":"Nucleic acids research"},{"issue":"33","key":"ref15","doi-asserted-by":"crossref","first-page":"27290","DOI":"10.1074\/jbc.M112.374777","article-title":"Stress-induced skeletal muscle Gadd45a expression reprograms myonuclei and causes muscle atrophy","volume":"287","author":"SM Ebert","year":"2012","journal-title":"The Journal of biological chemistry"},{"issue":"4","key":"ref16","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1210\/me.2009-0345","article-title":"The transcription factor ATF4 promotes skeletal myofiber atrophy during fasting","volume":"24","author":"SM Ebert","year":"2010","journal-title":"Molecular endocrinology (Baltimore, Md)"},{"issue":"5","key":"ref17","doi-asserted-by":"crossref","first-page":"2261","DOI":"10.3892\/or.2015.3845","article-title":"Muscle-specific E3 ubiquitin ligases are involved in muscle atrophy of cancer cachexia: an in vitro and in vivo study","volume":"33","author":"L Yuan","year":"2015","journal-title":"Oncology reports"},{"key":"ref18","article-title":"The role of E3 ubiquitin-ligases MuRF-1 and MAFbx in loss of skeletal muscle mass","author":"O Rom","year":"2015","journal-title":"Free radical biology & medicine"},{"issue":"1","key":"ref19","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1007\/s00441-013-1642-6","article-title":"Inhibition of the angiotensin-converting enzyme decreases skeletal muscle fibrosis in dystrophic mice by a diminution in the expression and activity of connective tissue growth factor (CTGF\/CCN-2)","volume":"353","author":"MG Morales","year":"2013","journal-title":"Cell and tissue research"},{"issue":"3","key":"ref20","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/0960-8966(94)90019-1","article-title":"Age-related changes in collagen gene expression in the muscles of mdx dystrophic and normal mice","volume":"4","author":"G Goldspink","year":"1994","journal-title":"Neuromuscular disorders: NMD"},{"issue":"5","key":"ref21","doi-asserted-by":"crossref","first-page":"R1173","DOI":"10.1152\/ajpregu.00735.2009","article-title":"Regulatory interactions between muscle and the immune system during muscle regeneration","volume":"298","author":"JG Tidball","year":"2010","journal-title":"American journal of physiology Regulatory, integrative and comparative physiology"},{"issue":"6","key":"ref22","doi-asserted-by":"crossref","first-page":"e65468","DOI":"10.1371\/journal.pone.0065468","article-title":"Omigapil treatment decreases fibrosis and improves respiratory rate in dy(2J) mouse model of congenital muscular dystrophy","volume":"8","author":"Q Yu","year":"2013","journal-title":"PloS one"},{"issue":"2","key":"ref23","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1002\/mus.21706","article-title":"Fibrosis inhibition and muscle histopathology improvement in laminin-alpha2-deficient mice","volume":"42","author":"Y Nevo","year":"2010","journal-title":"Muscle & nerve"},{"issue":"3","key":"ref24","doi-asserted-by":"crossref","first-page":"e18049","DOI":"10.1371\/journal.pone.0018049","article-title":"The Ras antagonist, farnesylthiosalicylic acid (FTS), decreases fibrosis and improves muscle strength in dy\/dy mouse model of muscular dystrophy","volume":"6","author":"Y Nevo","year":"2011","journal-title":"PloS one"},{"issue":"5","key":"ref25","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1002\/mus.21211","article-title":"Preclinical drug trials in the mdx mouse: assessment of reliable and sensitive outcome measures","volume":"39","author":"CF Spurney","year":"2009","journal-title":"Muscle & nerve"},{"issue":"43","key":"ref26","doi-asserted-by":"crossref","first-page":"13589","DOI":"10.1523\/JNEUROSCI.4286-09.2009","article-title":"Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease","volume":"29","author":"AL Southwell","year":"2009","journal-title":"The Journal of neuroscience: the official journal of the Society for Neuroscience"},{"issue":"8","key":"ref27","doi-asserted-by":"crossref","first-page":"3248","DOI":"10.1523\/JNEUROSCI.19-08-03248.1999","article-title":"Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation","volume":"19","author":"RJ Carter","year":"1999","journal-title":"The Journal of neuroscience: the official journal of the Society for Neuroscience"},{"issue":"3","key":"ref28","doi-asserted-by":"crossref","first-page":"E245","DOI":"10.1152\/ajpendo.00010.2014","article-title":"p53 and ATF4 mediate distinct and additive pathways to skeletal muscle atrophy during limb immobilization","volume":"307","author":"DK Fox","year":"2014","journal-title":"American journal of physiology Endocrinology and metabolism"},{"issue":"6335","key":"ref29","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1038\/352536a0","article-title":"The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy","volume":"352","author":"HH Stedman","year":"1991","journal-title":"Nature"},{"issue":"24","key":"ref30","doi-asserted-by":"crossref","first-page":"4891","DOI":"10.1093\/hmg\/ddr427","article-title":"Autophagy is increased in laminin alpha2 chain-deficient muscle and its inhibition improves muscle morphology in a mouse model of MDC1A","volume":"20","author":"V Carmignac","year":"2011","journal-title":"Human molecular genetics"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"e1690","DOI":"10.1038\/cddis.2015.60","article-title":"Life or death by NFkappaB, Losartan promotes survival in dy2J\/dy2J mouse of MDC1A","volume":"6","author":"M Elbaz","year":"2015","journal-title":"Cell death & disease"},{"issue":"4","key":"ref32","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1093\/ilar.43.4.244","article-title":"Guidelines for the design and statistical analysis of experiments using laboratory animals","volume":"43","author":"MF Festing","year":"2002","journal-title":"ILAR journal"},{"issue":"4","key":"ref33","doi-asserted-by":"crossref","first-page":"303","DOI":"10.4103\/0976-500X.119726","article-title":"How to calculate sample size in animal studies?","volume":"4","author":"J Charan","year":"2013","journal-title":"Journal of pharmacology & pharmacotherapeutics"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0197388","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,9]],"date-time":"2020-05-09T01:29:12Z","timestamp":1588987752000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0197388"}},"subtitle":[],"editor":[{"given":"Atsushi","family":"Asakura","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2018,5,15]]},"references-count":33,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2018,5,15]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0197388","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,5,15]]}}}