{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T12:34:14Z","timestamp":1778589254847,"version":"3.51.4"},"reference-count":93,"publisher":"Oxford University Press (OUP)","issue":"20","license":[{"start":{"date-parts":[[2019,7,26]],"date-time":"2019-07-26T00:00:00Z","timestamp":1564099200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"National Network of Excellence in Neuroscience, Teva Pharmaceutical Industries Ltd","award":["3031"],"award-info":[{"award-number":["3031"]}]},{"name":"National Network of Excellence in Neuroscience, Teva Pharmaceutical Industries Ltd","award":["1059"],"award-info":[{"award-number":["1059"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,10,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Congenital muscular dystrophy type-1A (Lama2-CMD) and Duchenne muscular dystrophy (DMD) result from deficiencies of laminin-\u03b12 and dystrophin proteins, respectively. Although both proteins strengthen the sarcolemma, they are implicated in clinically distinct phenotypes. We used RNA-deep sequencing (RNA-Seq) of dy2J\/dy2J, Lama2-CMD mouse model, skeletal muscle at 8\u00a0weeks of age to elucidate disease pathophysiology. This study is the first report of dy2J\/dy2J model whole transcriptome profile. RNA-Seq of the mdx mouse model of DMD and wild-type (WT) mouse was carried as well in order to enable a novel comparison of dy2J\/dy2J to mdx. A large group of shared differentially expressed genes (DEGs) was found in dy2J\/dy2J and mdx models (1834 common DEGs, false discovery rate [FDR]\u2009&lt;\u20090.05). Enrichment pathway analysis using ingenuity pathway analysis showed enrichment of inflammation, fibrosis, cellular movement, migration and proliferation of cells, apoptosis and necrosis in both mouse models (P-values 3E-10\u20139E-37). Via canonical pathway analysis, actin cytoskeleton, integrin, integrin-linked kinase, NF-kB, renin\u2013angiotensin, epithelial\u2013mesenchymal transition, and calcium signaling were also enriched and upregulated in both models (FDR\u2009&lt;\u20090.05). Interestingly, significant downregulation of Pax7 was detected in dy2J\/dy2J compared to upregulation of this key regeneration gene in mdx mice. Pax3 and Mamstr genes were also downregulated in dy2J\/dy2J compared to WT mice. These results may explain the distinct disease course and severity in these models. While the mdx model at that stage shows massive regeneration, the dy2J\/dy2J shows progressive dystrophic process. Our data deepen our understanding of the molecular pathophysiology and suggest new targets for additional therapies to upregulate regeneration in Lama2-CMD.<\/jats:p>","DOI":"10.1093\/hmg\/ddz180","type":"journal-article","created":{"date-parts":[[2019,7,24]],"date-time":"2019-07-24T11:12:03Z","timestamp":1563966723000},"page":"3369-3390","source":"Crossref","is-referenced-by-count":10,"title":["Pax7, Pax3 and Mamstr genes are involved in skeletal muscle impaired regeneration of dy2J\/dy2J mouse model of Lama2-CMD"],"prefix":"10.1093","volume":"28","author":[{"given":"Nurit","family":"Yanay","sequence":"first","affiliation":[{"name":"Felsenstein Medical Research Center, Tel-Aviv University, Tel-Aviv, Israel"},{"name":"Institute of Neurology, Schneider Children's Medical Center, Tel-Aviv University, Tel-Aviv, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Moran","family":"Elbaz","sequence":"additional","affiliation":[{"name":"Pediatric Neuromuscular Laboratory, Hadassah Hebrew University Medical Center, Jerusalem, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jenya","family":"Konikov-Rozenman","sequence":"additional","affiliation":[{"name":"Felsenstein Medical Research Center, Tel-Aviv University, Tel-Aviv, Israel"},{"name":"Institute of Neurology, Schneider Children's Medical Center, Tel-Aviv University, Tel-Aviv, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sharona","family":"Elgavish","sequence":"additional","affiliation":[{"name":"Info-CORE, I-CORE Bioinformatics Unit, The Hebrew University of Jerusalem and Hadassah Medical Center, Jerusalem, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuval","family":"Nevo","sequence":"additional","affiliation":[{"name":"Info-CORE, I-CORE Bioinformatics Unit, The Hebrew University of Jerusalem and Hadassah Medical Center, Jerusalem, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yakov","family":"Fellig","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hadassah Hebrew University Medical Center, Jerusalem, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Malcolm","family":"Rabie","sequence":"additional","affiliation":[{"name":"Felsenstein Medical Research Center, Tel-Aviv University, Tel-Aviv, Israel"},{"name":"Institute of Neurology, Schneider Children's Medical Center, Tel-Aviv University, Tel-Aviv, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stella","family":"Mitrani-Rosenbaum","sequence":"additional","affiliation":[{"name":"Goldyne Savad Institute of Gene Therapy, Hadassah Hebrew University Medical Center, Jerusalem, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoram","family":"Nevo","sequence":"additional","affiliation":[{"name":"Felsenstein Medical Research Center, Tel-Aviv University, Tel-Aviv, Israel"},{"name":"Institute of Neurology, Schneider Children's Medical Center, Tel-Aviv University, Tel-Aviv, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2019,7,26]]},"reference":[{"key":"2019120404004403500_ref1","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/S0959-437X(03)00048-0","article-title":"Muscular dystrophies: genes to pathogenesis","volume":"13","author":"Dalkilic","year":"2003","journal-title":"Curr. Opin. Genet. Dev."},{"key":"2019120404004403500_ref2","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1146\/annurev.pathol.2.010506.091936","article-title":"Muscle diseases: the muscular dystrophies","volume":"2","author":"McNally","year":"2007","journal-title":"Annu. Rev. Pathol."},{"key":"2019120404004403500_ref3","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1177\/1073858414558336","article-title":"Membrane injury and repair in the muscular dystrophies","volume":"21","author":"Cooper","year":"2015","journal-title":"Neuroscientist"},{"key":"2019120404004403500_ref4","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1146\/annurev.physiol.010908.163216","article-title":"Mechanisms of muscle degeneration, regeneration, and repair in the muscular dystrophies","volume":"71","author":"Wallace","year":"2009","journal-title":"Annu. Rev. Physiol."},{"key":"2019120404004403500_ref5","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":"Durbeej","year":"2015","journal-title":"Curr. Top Membr."},{"key":"2019120404004403500_ref6","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":"Gawlik","year":"2011","journal-title":"Skelet. Muscle"},{"key":"2019120404004403500_ref7","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1146\/annurev-genom-090314-025003","article-title":"The pathogenesis and therapy of muscular dystrophies","volume":"16","author":"Guiraud","year":"2015","journal-title":"Annu. Rev. Genomics Hum. Genet."},{"key":"2019120404004403500_ref8","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1542\/pir.21-7-233","article-title":"Muscular dystrophy","volume":"21","author":"Roland","year":"2000","journal-title":"Pediatr. Rev."},{"key":"2019120404004403500_ref9","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1002\/mus.1192","article-title":"The dystrophin\u2013glycoprotein complex, cellular signaling, and the regulation of cell survival in the muscular dystrophies","volume":"24","author":"Rando","year":"2001","journal-title":"Muscle Nerve"},{"key":"2019120404004403500_ref10","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":"Xu","year":"1994","journal-title":"Nat. Genet."},{"key":"2019120404004403500_ref11","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/S0960-8966(99)00056-5","article-title":"Identification of homozygous and heterozygous dy2J mice by PCR","volume":"10","author":"Vilquin","year":"2000","journal-title":"Neuromuscul. Disord."},{"key":"2019120404004403500_ref12","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1159\/000469192","article-title":"Molecular studies of progressive muscular dystrophy (Duchenne)","volume":"38","author":"Kunkel","year":"1987","journal-title":"Enzyme"},{"key":"2019120404004403500_ref13","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1242\/dmm.018424","article-title":"Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy","volume":"8","author":"McGreevy","year":"2015","journal-title":"Dis. Model. Mech."},{"key":"2019120404004403500_ref14","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.nano.2018.11.012","article-title":"Liposomal steroid nano-drug is superior to steroids as-is in mdx mouse model of Duchenne muscular dystrophy","volume":"16","author":"Turjeman","year":"2018","journal-title":"Nanomedicine"},{"key":"2019120404004403500_ref15","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1152\/physrev.00031.2010","article-title":"Fiber types in mammalian skeletal muscles","volume":"91","author":"Schiaffino","year":"2011","journal-title":"Physiol. Rev."},{"key":"2019120404004403500_ref16","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/s13395-015-0046-6","article-title":"Developmental myosins: expression patterns and functional significance","volume":"5","author":"Schiaffino","year":"2017","journal-title":"Skelet. Muscle"},{"key":"2019120404004403500_ref17","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1212\/WNL.0b013e318207afeb","article-title":"SPP1 genotype is a determinant of disease severity in Duchenne muscular dystrophy","volume":"76","author":"Pegoraro","year":"2011","journal-title":"Neurology"},{"key":"2019120404004403500_ref18","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/j.bbrc.2005.12.224","article-title":"Expression profiling of muscles from Fukuyama-type congenital muscular dystrophy and laminin-alpha 2 deficient congenital muscular dystrophy; is congenital muscular dystrophy a primary fibrotic disease?","volume":"342","author":"Taniguchi","year":"2006","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"2019120404004403500_ref19","doi-asserted-by":"crossref","first-page":"3283","DOI":"10.1093\/hmg\/11.26.3283","article-title":"Gene expression profiling in dysferlinopathies using a dedicated muscle microarray","volume":"11","author":"Campanaro","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref20","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s10974-013-9350-0","article-title":"Increased sarcolipin expression and decreased sarco (endo) plasmic reticulum Ca2+ uptake in skeletal muscles of mouse models of Duchenne muscular dystrophy","volume":"34","author":"Schneider","year":"2013","journal-title":"J. Muscle Res. Cell Motil."},{"issue":"450\u2013455","key":"2019120404004403500_ref21","first-page":"S451","article-title":"Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy","volume":"44","author":"Sarparanta","year":"2012","journal-title":"Nat. Genet."},{"key":"2019120404004403500_ref22","doi-asserted-by":"crossref","first-page":"6588","DOI":"10.1093\/hmg\/ddv363","article-title":"Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D","volume":"24","author":"Bengoechea","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref23","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1164\/rccm.200301-041OC","article-title":"Integrin alpha4beta1 regulates migration across basement membranes by lung fibroblasts: a role for phosphatase and tensin homologue deleted on chromosome 10","volume":"168","author":"White","year":"2003","journal-title":"Am. J. Respir. Crit. Care Med."},{"issue":"1","key":"2019120404004403500_ref24","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1002\/path.5215","article-title":"Fibroblast-specific integrin alpha V differentially regulates type 17 and type 2 driven inflammation and fibrosis","volume":"248","author":"Sciurba","year":"2018","journal-title":"J. Pathol"},{"key":"2019120404004403500_ref25","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1007\/s00441-009-0834-6","article-title":"Integrins","volume":"339","author":"Barczyk","year":"2009","journal-title":"Cell Tissue Res."},{"key":"2019120404004403500_ref26","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1186\/gb-2007-8-5-215","article-title":"The integrins","volume":"8","author":"Takada","year":"2007","journal-title":"Genome Biol."},{"key":"2019120404004403500_ref27","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1005457","article-title":"Runx1 transcription factor is required for myoblasts proliferation during muscle regeneration","volume":"11","author":"Umansky","year":"2015","journal-title":"PLoS Genet."},{"issue":"Pt 2","key":"2019120404004403500_ref28","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1242\/jcs.113.2.279","article-title":"Insulin-induced actin filament remodeling colocalizes actin with phosphatidylinositol 3-kinase and GLUT4 in L6 myotubes","volume":"113","author":"Khayat","year":"2000","journal-title":"J. Cell Sci."},{"key":"2019120404004403500_ref29","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1210\/me.2003-0294","article-title":"Skeletal muscle cells and adipocytes differ in their reliance on TC10 and Rac for insulin-induced actin remodeling","volume":"18","author":"JeBailey","year":"2004","journal-title":"Mol. Endocrinol."},{"key":"2019120404004403500_ref30","doi-asserted-by":"crossref","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":"Elbaz","year":"2015","journal-title":"Cell Death Dis."},{"key":"2019120404004403500_ref31","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1002\/ana.22694","article-title":"Losartan, a therapeutic candidate in congenital muscular dystrophy: studies in the dy(2J) \/dy(2J) mouse","volume":"71","author":"Elbaz","year":"2012","journal-title":"Ann. Neurol."},{"key":"2019120404004403500_ref32","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1186\/1471-2164-6-98","article-title":"Muscle regeneration in dystrophin-deficient mdx mice studied by gene expression profiling","volume":"6","author":"Turk","year":"2005","journal-title":"BMC Genomics"},{"key":"2019120404004403500_ref33","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1096\/fj.06-7285com","article-title":"Gene expression profiling in the early phases of DMD: a constant molecular signature characterizes DMD muscle from early postnatal life throughout disease progression","volume":"21","author":"Pescatori","year":"2007","journal-title":"FASEB J."},{"key":"2019120404004403500_ref34","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1093\/hmg\/11.3.263","article-title":"A chronic inflammatory response dominates the skeletal muscle molecular signature in dystrophin-deficient mdx mice","volume":"11","author":"Porter","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref35","doi-asserted-by":"crossref","first-page":"15000","DOI":"10.1073\/pnas.192571199","article-title":"Gene expression comparison of biopsies from Duchenne muscular dystrophy (DMD) and normal skeletal muscle","volume":"99","author":"Haslett","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2019120404004403500_ref36","doi-asserted-by":"crossref","first-page":"1813","DOI":"10.1093\/hmg\/ddg197","article-title":"Dissection of temporal gene expression signatures of affected and spared muscle groups in dystrophin-deficient (mdx) mice","volume":"12","author":"Porter","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref37","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1093\/hmg\/ddh033","article-title":"Temporal gene expression profiling of dystrophin-deficient (mdx) mouse diaphragm identifies conserved and muscle group-specific mechanisms in the pathogenesis of muscular dystrophy","volume":"13","author":"Porter","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref38","doi-asserted-by":"publisher","DOI":"10.1371\/currents.md.d0c203c018bc024f2f4c9791ecb05f88","article-title":"Exploratory profiling of urine microRNAs in the dy(2J)\/dy(2J) mouse model of LAMA2-CMD: relation to disease progression","volume":"10","author":"Moreira Soares Oliveira","year":"2018","journal-title":"PLoS Curr."},{"key":"2019120404004403500_ref39","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1152\/physiolgenomics.00226.2005","article-title":"Gene expression profiling of diaphragm muscle in alpha2-laminin (merosin)-deficient dy\/dy dystrophic mice","volume":"25","author":"van Lunteren","year":"2006","journal-title":"Physiol. Genomics"},{"key":"2019120404004403500_ref40","doi-asserted-by":"crossref","first-page":"3001","DOI":"10.1074\/mcp.M113.032276","article-title":"Quantitative proteomic analysis reveals metabolic alterations, calcium dysregulation, and increased expression of extracellular matrix proteins in laminin alpha2 chain-deficient muscle. Mol","volume":"13","author":"de Oliveira","year":"2014","journal-title":"Cell Proteomics"},{"key":"2019120404004403500_ref41","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1111\/j.1365-2559.2011.04051.x","article-title":"Osteopontin is highly expressed in severely dystrophic muscle and seems to play a role in muscle regeneration and fibrosis","volume":"59","author":"Zanotti","year":"2011","journal-title":"Histopathology"},{"key":"2019120404004403500_ref42","doi-asserted-by":"crossref","first-page":"44059","DOI":"10.1038\/srep44059","article-title":"Potent pro-inflammatory and pro-fibrotic molecules, osteopontin and galectin-3, are not major disease modulators of laminin alpha2 chain-deficient muscular dystrophy","volume":"7","author":"Gawlik","year":"2017","journal-title":"Sci. Rep."},{"key":"2019120404004403500_ref43","doi-asserted-by":"crossref","first-page":"241","DOI":"10.3233\/JND-140066","article-title":"Muscle-derived proteins as serum biomarkers for monitoring disease progression in three forms of muscular dystrophy","volume":"2","author":"Burch","year":"2016","journal-title":"J. Neuromuscul. Dis."},{"key":"2019120404004403500_ref44","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1093\/clinchem\/43.3.476","article-title":"Cardiac troponin T composition in normal and regenerating human skeletal muscle","volume":"43","author":"Bodor","year":"1997","journal-title":"Clin. Chem."},{"key":"2019120404004403500_ref45","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1161\/CIRCGENETICS.115.001322","article-title":"Elevated plasma cardiac troponin T levels caused by skeletal muscle damage in Pompe disease","volume":"9","author":"Wens","year":"2016","journal-title":"Circ. Cardiovasc. Genet."},{"key":"2019120404004403500_ref46","doi-asserted-by":"crossref","first-page":"4978","DOI":"10.1093\/hmg\/ddr433","article-title":"Expression of the dystrophin isoform Dp116 preserves functional muscle mass and extends lifespan without preventing dystrophy in severely dystrophic mice","volume":"20","author":"Judge","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref47","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1093\/hmg\/6.6.831","article-title":"Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice","volume":"6","author":"Williamson","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref48","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/1535370215597194","article-title":"Epithelial-mesenchymal transition: an emerging target in tissue fibrosis","volume":"241","author":"Li","year":"2013","journal-title":"Exp. Biol. Med. (Maywood)"},{"key":"2019120404004403500_ref49","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1016\/S0002-9440(10)63188-4","article-title":"Transforming growth factor-beta1 induces the differentiation of myogenic cells into fibrotic cells in injured skeletal muscle: a key event in muscle fibrogenesis","volume":"164","author":"Li","year":"2004","journal-title":"Am. J. Pathol."},{"key":"2019120404004403500_ref50","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/1471-2377-5-7","article-title":"Diseased muscles that lack dystrophin or laminin-alpha2 have altered compositions and proliferation of mononuclear cell populations","volume":"5","author":"Girgenrath","year":"2005","journal-title":"BMC Neurol."},{"key":"2019120404004403500_ref51","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1111\/apha.12099","article-title":"A novel GFP reporter mouse reveals Mustn1 expression in adult regenerating skeletal muscle, activated satellite cells and differentiating myoblasts","volume":"208","author":"Krause","year":"2013","journal-title":"Acta Physiol (Oxf.)"},{"key":"2019120404004403500_ref52","doi-asserted-by":"crossref","first-page":"C1100","DOI":"10.1152\/ajpcell.00553.2009","article-title":"Silencing of Mustn1 inhibits myogenic fusion and differentiation","volume":"298","author":"Liu","year":"2010","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2019120404004403500_ref53","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.ydbio.2004.08.015","article-title":"Pax-7 up-regulation inhibits myogenesis and cell cycle progression in satellite cells: a potential mechanism for self-renewal","volume":"275","author":"Olguin","year":"2004","journal-title":"Dev. Biol."},{"key":"2019120404004403500_ref54","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1083\/jcb.200312007","article-title":"Muscle satellite cells adopt divergent fates: a mechanism for self-renewal?","volume":"166","author":"Zammit","year":"2004","journal-title":"J. Cell Biol."},{"key":"2019120404004403500_ref55","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1242\/dev.01617","article-title":"Lineage analysis of the avian dermomyotome sheet reveals the existence of single cells with both dermal and muscle progenitor fates","volume":"132","author":"Ben-Yair","year":"2005","journal-title":"Development"},{"key":"2019120404004403500_ref56","doi-asserted-by":"crossref","first-page":"1426","DOI":"10.1101\/gad.345505","article-title":"Pax3\/Pax7 mark a novel population of primitive myogenic cells during development","volume":"19","author":"Kassar-Duchossoy","year":"2005","journal-title":"Genes Dev."},{"key":"2019120404004403500_ref57","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1083\/jcb.200508044","article-title":"Pax3 and Pax7 have distinct and overlapping functions in adult muscle progenitor cells","volume":"172","author":"Relaix","year":"2006","journal-title":"J. Cell Biol."},{"key":"2019120404004403500_ref58","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1038\/nature03594","article-title":"A Pax3\/Pax7-dependent population of skeletal muscle progenitor cells","volume":"435","author":"Relaix","year":"2005","journal-title":"Nature"},{"key":"2019120404004403500_ref59","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1016\/j.devcel.2012.03.014","article-title":"Transcriptional dominance of Pax7 in adult myogenesis is due to high-affinity recognition of homeodomain motifs","volume":"22","author":"Soleimani","year":"2012","journal-title":"Dev. Cell"},{"key":"2019120404004403500_ref60","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/S1534-5807(02)00254-X","article-title":"The regulation of notch signaling controls satellite cell activation and cell fate determination in postnatal myogenesis","volume":"3","author":"Conboy","year":"2002","journal-title":"Dev. Cell"},{"key":"2019120404004403500_ref61","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1083\/jcb.200508001","article-title":"Distinct roles for Pax7 and Pax3 in adult regenerative myogenesis","volume":"172","author":"Kuang","year":"2006","journal-title":"J. Cell Biol."},{"key":"2019120404004403500_ref62","doi-asserted-by":"crossref","first-page":"C155","DOI":"10.1152\/ajpcell.00269.2016","article-title":"Renin\u2013angiotensin\u2013aldosterone system inhibitors improve membrane stability and change gene-expression profiles in dystrophic skeletal muscles","volume":"312","author":"Chadwick","year":"2016","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2019120404004403500_ref63","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0112477","article-title":"Non-invasive MRI and spectroscopy of mdx mice reveal temporal changes in dystrophic muscle imaging and in energy deficits","volume":"9","author":"Heier","year":"2014","journal-title":"PLoS One"},{"key":"2019120404004403500_ref64","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1093\/hmg\/ddt428","article-title":"Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy","volume":"23","author":"Van Ry","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref65","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1038\/s41467-017-01486-4","article-title":"Downregulation of myostatin pathway in neuromuscular diseases may explain challenges of anti-myostatin therapeutic approaches","volume":"8","author":"Mariot","year":"2017","journal-title":"Nat. Commun."},{"key":"2019120404004403500_ref66","doi-asserted-by":"crossref","DOI":"10.1098\/rstb.2016.0528","article-title":"Therapeutic potential of heat shock protein induction for muscular dystrophy and other muscle wasting conditions","volume":"373","author":"Thakur","year":"2018","journal-title":"Philos. Trans. R. Soc. Lond. B Biol. Sci."},{"key":"2019120404004403500_ref67","article-title":"Omigapil treatment decreases fibrosis and improves respiratory rate in dy(2J) mouse model of congenital muscular dystrophy","volume":"8","author":"Yu","year":"2013","journal-title":"PLoS One"},{"key":"2019120404004403500_ref68","first-page":"4624","article-title":"IGF-1\/GH axis enhances losartan treatment in Lama2-related muscular dystrophy","volume":"25","author":"Accorsi","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref69","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":"Nevo","year":"2010","journal-title":"Muscle Nerve"},{"key":"2019120404004403500_ref70","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1016\/j.stemcr.2015.04.007","article-title":"Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy","volume":"4","author":"Pessina","year":"2015","journal-title":"Stem Cell Rep."},{"key":"2019120404004403500_ref71","doi-asserted-by":"crossref","first-page":"4131","DOI":"10.1111\/febs.12338","article-title":"Interleukin-6 myokine signaling in skeletal muscle: a double-edged sword?","volume":"280","author":"Munoz-Canoves","year":"2013","journal-title":"FEBS J."},{"key":"2019120404004403500_ref72","doi-asserted-by":"crossref","first-page":"27","DOI":"10.3389\/fnut.2015.00027","article-title":"Post-exercise skeletal muscle glycogen related to plasma cytokines and muscle IL-6 protein content, but not muscle cytokine mRNA expression","volume":"2","author":"Nieman","year":"2015","journal-title":"Front. Nutr."},{"key":"2019120404004403500_ref73","doi-asserted-by":"crossref","first-page":"23823","DOI":"10.1074\/jbc.M112.449074","article-title":"Angiotensin II inhibits satellite cell proliferation and prevents skeletal muscle regeneration","volume":"288","author":"Yoshida","year":"2013","journal-title":"J. Biol. Chem."},{"key":"2019120404004403500_ref74","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1186\/2044-5040-2-18","article-title":"Angiotensin II type 1 receptor antagonists alleviate muscle pathology in the mouse model for laminin-alpha2-deficient congenital muscular dystrophy (MDC1A)","volume":"2","author":"Meinen","year":"2012","journal-title":"Skelet. Muscle"},{"key":"2019120404004403500_ref75","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/nm1536","article-title":"Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states","volume":"13","author":"Cohn","year":"2007","journal-title":"Nat. Med."},{"key":"2019120404004403500_ref76","doi-asserted-by":"crossref","first-page":"2611","DOI":"10.1016\/j.ajpath.2011.02.024","article-title":"Inhibiting TGF-beta activity improves respiratory function in mdx mice","volume":"178","author":"Nelson","year":"2011","journal-title":"Am. J. Pathol."},{"key":"2019120404004403500_ref77","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1038\/ncb0203-87","article-title":"Signalling pathways that mediate skeletal muscle hypertrophy and atrophy","volume":"5","author":"Glass","year":"2003","journal-title":"Nat. Cell Biol."},{"key":"2019120404004403500_ref78","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1038\/nm0604-584","article-title":"Balancing muscle hypertrophy and atrophy","volume":"10","author":"Hoffman","year":"2004","journal-title":"Nat. Med."},{"key":"2019120404004403500_ref79","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1083\/jcb.200707175","article-title":"Integrin-linked kinase stabilizes myotendinous junctions and protects muscle from stress-induced damage","volume":"180","author":"Wang","year":"2008","journal-title":"J. Cell Biol."},{"key":"2019120404004403500_ref80","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1074\/mcp.M112.023127","article-title":"Identification of disease specific pathways using in vivo SILAC proteomics in dystrophin deficient mdx mouse. Mol","volume":"12","author":"Rayavarapu","year":"2013","journal-title":"Cell Proteomics"},{"key":"2019120404004403500_ref81","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.ajpath.2010.11.027","article-title":"Long-term blocking of calcium channels in mdx mice results in differential effects on heart and skeletal muscle","volume":"178","author":"Jorgensen","year":"2011","journal-title":"Am. J. Pathol."},{"key":"2019120404004403500_ref82","doi-asserted-by":"crossref","first-page":"45272","DOI":"10.1038\/srep45272","article-title":"Bioenergetic impairment in congenital muscular dystrophy type 1A and Leigh syndrome muscle cells","volume":"7","author":"Fontes-Oliveira","year":"2017","journal-title":"Sci. Rep."},{"key":"2019120404004403500_ref83","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1002\/mus.21188","article-title":"Diltiazem and verapamil protect dystrophin-deficient muscle fibers of MDX mice from degeneration: a potential role in calcium buffering and sarcolemmal stability","volume":"39","author":"Matsumura","year":"2009","journal-title":"Muscle Nerve"},{"key":"2019120404004403500_ref84","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1139\/Y09-058","article-title":"Calcium and the damage pathways in muscular dystrophy","volume":"88","author":"Allen","year":"2010","journal-title":"Can. J. Physiol. Pharmacol."},{"key":"2019120404004403500_ref85","doi-asserted-by":"crossref","first-page":"1549","DOI":"10.1002\/(SICI)1097-4598(199612)19:12<1549::AID-MUS4>3.0.CO;2-A","article-title":"The mdx-amplification-resistant mutation system assay, a simple and rapid polymerase chain reaction-based detection of the mdx allele","volume":"19","author":"Amalfitano","year":"1996","journal-title":"Muscle Nerve"},{"key":"2019120404004403500_ref86","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":"Sunada","year":"1995","journal-title":"Hum. Mol. Genet."},{"key":"2019120404004403500_ref87","doi-asserted-by":"crossref","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":"Nevo","year":"2011","journal-title":"PLoS One"},{"key":"2019120404004403500_ref88","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1093\/bioinformatics\/btv566","article-title":"Qualimap 2: advanced multi-sample quality control for high-throughput sequencing data","volume":"32","author":"Okonechnikov","year":"2015","journal-title":"Bioinformatics"},{"key":"2019120404004403500_ref89","doi-asserted-by":"crossref","first-page":"R36","DOI":"10.1186\/gb-2013-14-4-r36","article-title":"TopHat2: accurate alignment of transcriptomes in the presence of insertions, deletions and gene fusions","volume":"14","author":"Kim","year":"2013","journal-title":"Genome Biol."},{"key":"2019120404004403500_ref90","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1038\/nbt.1621","article-title":"Transcript assembly and quantification by RNA-Seq reveals unannotated transcripts and isoform switching during cell differentiation","volume":"28","author":"Trapnell","year":"2010","journal-title":"Nat. Biotechnol."},{"key":"2019120404004403500_ref91","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1038\/nbt.2450","article-title":"Differential analysis of gene regulation at transcript resolution with RNA-seq","volume":"31","author":"Trapnell","year":"2013","journal-title":"Nat. Biotechnol."},{"key":"2019120404004403500_ref92","doi-asserted-by":"crossref","first-page":"3387","DOI":"10.1073\/pnas.1222643110","article-title":"Long noncoding RNAs regulate adipogenesis","volume":"110","author":"Sun","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2019120404004403500_ref93","doi-asserted-by":"crossref","first-page":"D991","DOI":"10.1093\/nar\/gks1193","article-title":"NCBI GEO: archive for functional genomics data sets--update","volume":"41","author":"Barrett","year":"2013","journal-title":"Nucleic Acids Res."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/advance-article-pdf\/doi\/10.1093\/hmg\/ddz180\/30155920\/ddz180.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/28\/20\/3369\/31192065\/ddz180.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/28\/20\/3369\/31192065\/ddz180.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T13:49:43Z","timestamp":1695044983000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/28\/20\/3369\/5538901"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7,26]]},"references-count":93,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2019,7,26]]},"published-print":{"date-parts":[[2019,10,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddz180","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2019,10,15]]},"published":{"date-parts":[[2019,7,26]]}}}