{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:23:20Z","timestamp":1792787000065,"version":"4.5.0"},"reference-count":112,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T00:00:00Z","timestamp":1538352000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T00:00:00Z","timestamp":1538352000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R01-DK36425"],"award-info":[{"award-number":["R01-DK36425"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Cantons of Basel-Stadt"},{"name":"Basel-Landschaft"},{"DOI":"10.13039\/100010767","name":"Swiss Foundation for Research on Muscle Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010767","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Association Francaise contre les Myopathies","id":[{"id":"https:\/\/ror.org\/0162y2387","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100008038","name":"Neuromuscular Research Association Basel","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008038","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Matrix Biology"],"published-print":{"date-parts":[[2018,10]]},"DOI":"10.1016\/j.matbio.2017.11.009","type":"journal-article","created":{"date-parts":[[2017,11,27]],"date-time":"2017-11-27T13:01:57Z","timestamp":1511787717000},"page":"174-187","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":101,"special_numbering":"C","title":["Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies"],"prefix":"10.1016","volume":"71-72","author":[{"given":"Peter D.","family":"Yurchenco","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen K.","family":"McKee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith R.","family":"Reinhard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4974-9384","authenticated-orcid":false,"given":"Markus A.","family":"R\u00fcegg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.matbio.2017.11.009_bb0005","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.2174\/138161209787846766","article-title":"Developmental and pathogenic mechanisms of basement membrane assembly","volume":"15","author":"Yurchenco","year":"2009","journal-title":"Curr. Pharm. Des."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0010","doi-asserted-by":"crossref","first-page":"a004911","DOI":"10.1101\/cshperspect.a004911","article-title":"Basement membranes: cell scaffoldings and signaling platforms","volume":"3","author":"Yurchenco","year":"2011","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0015","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.matbio.2016.12.009","article-title":"The nature and biology of basement membranes","volume":"57-58","author":"Pozzi","year":"2017","journal-title":"Matrix Biol."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0020","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.jsb.2010.02.004","article-title":"Laminin chain assembly is regulated by specific coiled-coil interactions","volume":"170","author":"Macdonald","year":"2010","journal-title":"J. Struct. Biol."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0025","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.matbio.2005.05.006","article-title":"A simplified laminin nomenclature","volume":"24","author":"Aumailley","year":"2005","journal-title":"Matrix Biol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0030","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1111\/j.1749-6632.1998.tb10133.x","article-title":"The targeted deletion of the LAMC1 gene","volume":"857","author":"Smyth","year":"1998","journal-title":"Ann. N. Y. Acad. Sci."},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0035","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1083\/jcb.201609037","article-title":"Integrin-mediated mechanotransduction","volume":"215","author":"Sun","year":"2016","journal-title":"J. Cell Biol."},{"issue":"7\u20138","key":"10.1016\/j.matbio.2017.11.009_bb0040","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1007\/s00018-004-4510-4","article-title":"Congenital muscular dystrophy: molecular and cellular aspects","volume":"62","author":"Jimenez-Mallebrera","year":"2005","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1016\/j.matbio.2017.11.009_bb0045","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.matbio.2016.07.009","article-title":"Molecular pathology of the basement membrane zone in heritable blistering diseases: the paradigm of epidermolysis bullosa","volume":"57-58","author":"Uitto","year":"2017","journal-title":"Matrix Biol."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0050","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1002\/(SICI)1097-0177(200006)218:2<213::AID-DVDY1>3.0.CO;2-R","article-title":"Form and function: the laminin family of heterotrimers","volume":"218","author":"Colognato","year":"2000","journal-title":"Dev. Dyn."},{"issue":"22","key":"10.1016\/j.matbio.2017.11.009_bb0055","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1016\/S0960-9822(00)80056-1","article-title":"The laminin alpha2 expressed by dystrophic dy(2J) mice is defective in its ability to form polymers","volume":"9","author":"Colognato","year":"1999","journal-title":"Curr. Biol."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0060","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1172\/JCI90854","article-title":"Chimeric protein repair of laminin polymerization ameliorates muscular dystrophy phenotype","volume":"127","author":"McKee","year":"2017","journal-title":"J. Clin. Invest."},{"issue":"9","key":"10.1016\/j.matbio.2017.11.009_bb0065","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1016\/j.nmd.2017.06.008","article-title":"Congenital muscular dystrophies in the UK population: clinical and molecular spectrum of a large cohort diagnosed over a 12-year period","volume":"27","author":"Sframeli","year":"2017","journal-title":"Neuromuscul. Disord."},{"issue":"12","key":"10.1016\/j.matbio.2017.11.009_bb0070","first-page":"1601","article-title":"Activation of the lama2 gene in muscle regeneration: abortive regeneration in laminin alpha2-deficiency","volume":"79","author":"Kuang","year":"1999","journal-title":"Lab. Investig."},{"issue":"8","key":"10.1016\/j.matbio.2017.11.009_bb0075","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1002\/emmm.201100151","article-title":"Apoptosis inhibitors and mini-agrin have additive benefits in congenital muscular dystrophy mice","volume":"3","author":"Meinen","year":"2011","journal-title":"EMBO Mol. Med."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0080","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1177\/088307389501000610","article-title":"Demyelinating peripheral neuropathy in merosin-deficient congenital muscular dystrophy","volume":"10","author":"Shorer","year":"1995","journal-title":"J. Child Neurol."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0085","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0960-8966(96)00383-5","article-title":"Sequential study of central and peripheral nervous system involvement in an infant with merosin-deficient congenital muscular dystrophy","volume":"6","author":"Mercuri","year":"1996","journal-title":"Neuromuscul. Disord."},{"issue":"8","key":"10.1016\/j.matbio.2017.11.009_bb0090","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/j.nmd.2014.05.008","article-title":"Limb girdle muscular dystrophy due to LAMA2 mutations: diagnostic difficulties due to associated peripheral neuropathy","volume":"24","author":"Chan","year":"2014","journal-title":"Neuromuscul. Disord."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0095","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1177\/0883073807303219","article-title":"Merosin-negative congenital muscular dystrophy: diffusion-weighted imaging findings of brain","volume":"22","author":"Alkan","year":"2007","journal-title":"J. Child Neurol."},{"issue":"11","key":"10.1016\/j.matbio.2017.11.009_bb0100","doi-asserted-by":"crossref","first-page":"2084","DOI":"10.1212\/WNL.45.11.2084","article-title":"Merosin-negative congenital muscular dystrophy associated with extensive brain abnormalities","volume":"45","author":"Sunada","year":"1995","journal-title":"Neurology"},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0105","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/S0960-8966(96)00421-X","article-title":"Late onset muscular dystrophy with cerebral white matter changes due to partial merosin deficiency","volume":"7","author":"Tan","year":"1997","journal-title":"Neuromuscul. Disord."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0110","first-page":"324","article-title":"MR imaging findings in children with merosin-deficient congenital muscular dystrophy","volume":"20","author":"Caro","year":"1999","journal-title":"AJNR Am. J. Neuroradiol."},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0115","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.nmd.2013.12.011","article-title":"Diagnostic approach to the congenital muscular dystrophies","volume":"24","author":"Bonnemann","year":"2014","journal-title":"Neuromuscul. Disord."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0120","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1038\/embor.2011.3","article-title":"Determinants of laminin polymerization revealed by the structure of the alpha5 chain amino-terminal region","volume":"12","author":"Hussain","year":"2011","journal-title":"EMBO Rep."},{"issue":"7","key":"10.1016\/j.matbio.2017.11.009_bb0125","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0042473","article-title":"Crystal structures of the network-forming short-arm tips of the laminin beta1 and gamma1 chains","volume":"7","author":"Carafoli","year":"2012","journal-title":"PLoS One"},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0130","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1093\/hmg\/6.5.747","article-title":"Mild congenital muscular dystrophy in two patients with an internally deleted laminin alpha2-chain","volume":"6","author":"Allamand","year":"1997","journal-title":"Hum. Mol. Genet."},{"issue":"12","key":"10.1016\/j.matbio.2017.11.009_bb0135","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1073\/pnas.41.12.1079","article-title":"Dystrophia muscularis: a hereditary primary myopathy in the house mouse","volume":"41","author":"Michelson","year":"1955","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0140","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/0024-3205(70)90306-1","article-title":"Muscular dystrophy in the mouse caused by an allele at the dy-locus","volume":"9","author":"Meier","year":"1970","journal-title":"Life Sci."},{"issue":"Pt. 10","key":"10.1016\/j.matbio.2017.11.009_bb0145","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1242\/jcs.015354","article-title":"A single point mutation in the LN domain of LAMA2 causes muscular dystrophy and peripheral amyelination","volume":"121","author":"Patton","year":"2008","journal-title":"J. Cell Sci."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0150","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1006\/excr.1998.4025","article-title":"Disruption of the lama2 gene in embryonic stem cells: laminin alpha 2 is necessary for sustenance of mature muscle cells","volume":"241","author":"Kuang","year":"1998","journal-title":"Exp. Cell Res."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0155","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":"Miyagoe","year":"1997","journal-title":"FEBS Lett."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0160","doi-asserted-by":"crossref","first-page":"115","DOI":"10.3233\/JND-170217","article-title":"Improving reproducibility of phenotypic assessments in the DyW mouse model of laminin-alpha2 related congenital muscular dystrophy","volume":"4","author":"Willmann","year":"2017","journal-title":"J. Neuromuscul. Dis."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0165","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/s0960-8966(02)00266-3","article-title":"Laminin alpha2 deficiency and muscular dystrophy; genotype-phenotype correlation in mutant mice","volume":"13","author":"Guo","year":"2003","journal-title":"Neuromuscul. Disord."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0170","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."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0175","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":"10.1016\/j.matbio.2017.11.009_bb0180","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1083\/jcb.200411158","article-title":"Coordinate control of axon defasciculation and myelination by laminin-2 and -8","volume":"168","author":"Yang","year":"2005","journal-title":"J. Cell Biol."},{"issue":"46","key":"10.1016\/j.matbio.2017.11.009_bb0185","doi-asserted-by":"crossref","first-page":"15260","DOI":"10.1523\/JNEUROSCI.3678-13.2014","article-title":"The extracellular matrix protein laminin alpha2 regulates the maturation and function of the blood-brain barrier","volume":"34","author":"Menezes","year":"2014","journal-title":"J. Neurosci."},{"key":"10.1016\/j.matbio.2017.11.009_bb0190","doi-asserted-by":"crossref","first-page":"3413","DOI":"10.1038\/ncomms4413","article-title":"Astrocytic laminin regulates pericyte differentiation and maintains blood brain barrier integrity","volume":"5","author":"Yao","year":"2014","journal-title":"Nat. Commun."},{"issue":"1\u20132","key":"10.1016\/j.matbio.2017.11.009_bb0195","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0022-510X(01)00559-7","article-title":"Laminin alpha2 (merosin)-deficient muscular dystrophy and demyelinating neuropathy in two cats","volume":"189","author":"O'Brien","year":"2001","journal-title":"J. Neurol. Sci."},{"issue":"17","key":"10.1016\/j.matbio.2017.11.009_bb0200","doi-asserted-by":"crossref","first-page":"7092","DOI":"10.1073\/pnas.0700942104","article-title":"The zebrafish candyfloss mutant implicates extracellular matrix adhesion failure in laminin alpha2-deficient congenital muscular dystrophy","volume":"104","author":"Hall","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0205","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1111\/j.1939-1676.2001.tb02317.x","article-title":"Muscular dystrophy in female dogs","volume":"15","author":"Shelton","year":"2001","journal-title":"J. Vet. Intern. Med."},{"issue":"7","key":"10.1016\/j.matbio.2017.11.009_bb0210","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1083\/jcb.200203073","article-title":"Matrix assembly, regulation, and survival functions of laminin and its receptors in embryonic stem cell differentiation","volume":"157","author":"Li","year":"2002","journal-title":"J. Cell Biol."},{"issue":"29","key":"10.1016\/j.matbio.2017.11.009_bb0215","doi-asserted-by":"crossref","first-page":"21437","DOI":"10.1074\/jbc.M702963200","article-title":"Role of laminin terminal globular domains in basement membrane assembly","volume":"282","author":"McKee","year":"2007","journal-title":"J. Biol. Chem."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0220","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1083\/jcb.200501098","article-title":"Laminin-sulfatide binding initiates basement membrane assembly and enables receptor signaling in Schwann cells and fibroblasts","volume":"169","author":"Li","year":"2005","journal-title":"J. Cell Biol."},{"issue":"15","key":"10.1016\/j.matbio.2017.11.009_bb0225","doi-asserted-by":"crossref","first-page":"5723","DOI":"10.1073\/pnas.1323842111","article-title":"Microtubule binding distinguishes dystrophin from utrophin","volume":"111","author":"Belanto","year":"2014","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"7","key":"10.1016\/j.matbio.2017.11.009_bb0230","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1083\/jcb.200604179","article-title":"Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan","volume":"176","author":"Rezniczek","year":"2007","journal-title":"J. Cell Biol."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0235","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.matbio.2005.12.001","article-title":"Ligand-binding specificities of laminin-binding integrins: a comprehensive survey of laminin-integrin interactions using recombinant alpha3beta1, alpha6beta1, alpha7beta1 and alpha6beta4 integrins","volume":"25","author":"Nishiuchi","year":"2006","journal-title":"Matrix Biol."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0240","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1111\/j.1432-1033.1997.00138.x","article-title":"Localization of heparin binding activity in recombinant laminin G domain","volume":"250","author":"Sung","year":"1997","journal-title":"Eur. J. Biochem."},{"issue":"15","key":"10.1016\/j.matbio.2017.11.009_bb0245","doi-asserted-by":"crossref","first-page":"11144","DOI":"10.1074\/jbc.M609402200","article-title":"The requirement of the glutamic acid residue at the third position from the carboxyl termini of the laminin gamma chains in integrin binding by laminins","volume":"282","author":"Ido","year":"2007","journal-title":"J. Biol. Chem."},{"issue":"10","key":"10.1016\/j.matbio.2017.11.009_bb0250","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1038\/nchembio.2146","article-title":"Structural basis of laminin binding to the LARGE glycans on dystroglycan","volume":"12","author":"Briggs","year":"2016","journal-title":"Nat. Chem. Biol."},{"issue":"7","key":"10.1016\/j.matbio.2017.11.009_bb0255","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1016\/j.cell.2004.06.003","article-title":"Molecular recognition by LARGE is essential for expression of functional dystroglycan","volume":"117","author":"Kanagawa","year":"2004","journal-title":"Cell"},{"issue":"6064","key":"10.1016\/j.matbio.2017.11.009_bb0260","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1126\/science.1214115","article-title":"Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE","volume":"335","author":"Inamori","year":"2012","journal-title":"Science"},{"issue":"38","key":"10.1016\/j.matbio.2017.11.009_bb0265","doi-asserted-by":"crossref","first-page":"23418","DOI":"10.1074\/jbc.271.38.23418","article-title":"Dystroglycan is a dual receptor for agrin and laminin-2 in Schwann cell membrane","volume":"271","author":"Yamada","year":"1996","journal-title":"J. Biol. Chem."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0270","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1074\/jbc.273.1.600","article-title":"Agrin is a high-affinity binding protein of dystroglycan in non-muscle tissue","volume":"273","author":"Gesemann","year":"1998","journal-title":"J. Biol. Chem."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0275","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1016\/S1097-2765(00)80388-3","article-title":"The crystal structure of a laminin G-like module reveals the molecular basis of alpha-dystroglycan binding to laminins, perlecan, and agrin","volume":"4","author":"Hohenester","year":"1999","journal-title":"Mol. Cell"},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0280","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1093\/emboj\/18.4.863","article-title":"Binding of the G domains of laminin alpha1 and alpha2 chains and perlecan to heparin, sulfatides, alpha-dystroglycan and several extracellular matrix proteins","volume":"18","author":"Talts","year":"1999","journal-title":"EMBO J."},{"issue":"31","key":"10.1016\/j.matbio.2017.11.009_bb0285","doi-asserted-by":"crossref","first-page":"12573","DOI":"10.1073\/pnas.0906545106","article-title":"Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan","volume":"106","author":"Han","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"396","key":"10.1016\/j.matbio.2017.11.009_bb0290","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aal4649","article-title":"Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice","volume":"9","author":"Reinhard","year":"2017","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.matbio.2017.11.009_bb0295","doi-asserted-by":"crossref","first-page":"7636","DOI":"10.1016\/S0021-9258(17)39656-4","article-title":"Laminin polymerization in vitro. Evidence for a two-step assembly with domain specificity","volume":"260","author":"Yurchenco","year":"1985","journal-title":"J. Biol. Chem."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0300","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1083\/jcb.117.5.1119","article-title":"Laminin forms an independent network in basement membranes","volume":"117","author":"Yurchenco","year":"1992","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0305","doi-asserted-by":"crossref","first-page":"17286","DOI":"10.1016\/S0021-9258(19)85334-6","article-title":"Self-assembly and calcium-binding sites in laminin. A three-arm interaction model","volume":"268","author":"Yurchenco","year":"1993","journal-title":"J. Biol. Chem."},{"issue":"50","key":"10.1016\/j.matbio.2017.11.009_bb0310","doi-asserted-by":"crossref","first-page":"31525","DOI":"10.1074\/jbc.272.50.31525","article-title":"Self-assembly of laminin isoforms","volume":"272","author":"Cheng","year":"1997","journal-title":"J. Biol. Chem."},{"issue":"Pt 1","key":"10.1016\/j.matbio.2017.11.009_bb0315","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1042\/bj3620213","article-title":"Complete sequence, recombinant analysis and binding to laminins and sulphated ligands of the N-terminal domains of laminin [alpha]3B and [alpha]5 chains","volume":"362","author":"Garbe","year":"2002","journal-title":"Biochem. J."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0320","doi-asserted-by":"crossref","first-page":"1507","DOI":"10.1083\/jcb.139.6.1507","article-title":"Distribution and function of laminins in the neuromuscular system of developing, adult, and mutant mice","volume":"139","author":"Patton","year":"1997","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0325","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.matbio.2016.08.008","article-title":"The role of laminins in the organization and function of neuromuscular junctions","volume":"57\u201358","author":"Rogers","year":"2017","journal-title":"Matrix Biol."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0330","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1002\/(SICI)1096-9896(199909)189:1<4::AID-PATH332>3.0.CO;2-P","article-title":"The role of CD146 (Mel-CAM) in biology and pathology","volume":"189","author":"Shih","year":"1999","journal-title":"J. Pathol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0335","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.matbio.2014.06.002","article-title":"Laminins 411 and 421 differentially promote tumor cell migration via alpha6beta1 integrin and MCAM (CD146)","volume":"38","author":"Ishikawa","year":"2014","journal-title":"Matrix Biol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0340","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.matbio.2014.03.003","article-title":"Monoclonal antibodies to human laminin alpha4 chain globular domain inhibit tumor cell adhesion and migration on laminins 411 and 421, and binding of alpha6beta1 integrin and MCAM to alpha4-laminins","volume":"36","author":"Ishikawa","year":"2014","journal-title":"Matrix Biol."},{"issue":"45","key":"10.1016\/j.matbio.2017.11.009_bb0345","doi-asserted-by":"crossref","first-page":"35192","DOI":"10.1074\/jbc.M003261200","article-title":"Structural and functional analysis of the recombinant G domain of the laminin {alpha}4 chain and its proteolytic processing in tissues","volume":"275","author":"Talts","year":"2000","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.matbio.2017.11.009_bb0350","doi-asserted-by":"crossref","first-page":"3137","DOI":"10.1002\/j.1460-2075.1991.tb04875.x","article-title":"Recombinant nidogen consists of three globular domains and mediates binding of laminin to collagen type IV","volume":"10","author":"Fox","year":"1991","journal-title":"EMBO J."},{"issue":"19","key":"10.1016\/j.matbio.2017.11.009_bb0355","doi-asserted-by":"crossref","first-page":"5154","DOI":"10.1002\/j.1460-2075.1996.tb00899.x","article-title":"Site-directed mutagenesis and structural interpretation of the nidogen binding site of the laminin gamma1 chain","volume":"15","author":"Poschl","year":"1996","journal-title":"EMBO J."},{"issue":"6\u20137","key":"10.1016\/j.matbio.2017.11.009_bb0360","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/S0960-8966(99)00033-4","article-title":"Distribution of ten laminin chains in dystrophic and regenerating muscles","volume":"9","author":"Patton","year":"1999","journal-title":"Neuromuscul. Disord."},{"issue":"6853","key":"10.1016\/j.matbio.2017.11.009_bb0365","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1038\/35095054","article-title":"An agrin minigene rescues dystrophic symptoms in a mouse model for congenital muscular dystrophy","volume":"413","author":"Moll","year":"2001","journal-title":"Nature"},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0370","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1006\/excr.1998.4244","article-title":"Expression of laminin alpha1, alpha2, alpha4, and alpha5 chains, fibronectin, and tenascin-C in skeletal muscle of dystrophic 129ReJ dy\/dy mice","volume":"246","author":"Ringelmann","year":"1999","journal-title":"Exp. Cell Res."},{"issue":"Pt 2","key":"10.1016\/j.matbio.2017.11.009_bb0375","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1042\/bj20021500","article-title":"Beta1 integrin and alpha-dystroglycan binding sites are localized to different laminin-G-domain-like (LG) modules within the laminin alpha5 chain G domain","volume":"371","author":"Yu","year":"2003","journal-title":"Biochem. J."},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0380","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1172\/JCI3705","article-title":"Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models","volume":"102","author":"Kuang","year":"1998","journal-title":"J. Clin. Invest."},{"issue":"16","key":"10.1016\/j.matbio.2017.11.009_bb0385","doi-asserted-by":"crossref","first-page":"1775","DOI":"10.1093\/hmg\/ddh190","article-title":"Laminin {alpha}1 chain reduces muscular dystrophy in laminin {alpha}2 chain deficient mice","volume":"13","author":"Gawlik","year":"2004","journal-title":"Hum. Mol. Genet."},{"issue":"18","key":"10.1016\/j.matbio.2017.11.009_bb0390","doi-asserted-by":"crossref","first-page":"2690","DOI":"10.1093\/hmg\/ddl201","article-title":"Laminin alpha1 chain improves laminin alpha2 chain deficient peripheral neuropathy","volume":"15","author":"Gawlik","year":"2006","journal-title":"Hum. Mol. Genet."},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0395","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1016\/j.ajpath.2011.12.019","article-title":"Laminin-111 protein therapy reduces muscle pathology and improves viability of a mouse model of merosin-deficient congenital muscular dystrophy","volume":"180","author":"Rooney","year":"2012","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.matbio.2017.11.009_bb0400","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.omtn.2016.11.004","article-title":"Increased expression of laminin subunit alpha 1 chain by dCas9-VP160","volume":"6","author":"Perrin","year":"2017","journal-title":"Mol. Ther.\u2013Nucleic Acids"},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0405","doi-asserted-by":"crossref","first-page":"1728","DOI":"10.1016\/j.ajpath.2010.12.030","article-title":"Transgenic expression of laminin alpha1 chain does not prevent muscle disease in the mdx mouse model for Duchenne muscular dystrophy","volume":"178","author":"Gawlik","year":"2011","journal-title":"Am. J. Pathol."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0410","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1089\/nat.2017.0682","article-title":"Development of exon skipping therapies for Duchenne muscular dystrophy: a critical review and a perspective on the outstanding issues","volume":"27","author":"Aartsma-Rus","year":"2017","journal-title":"Nucleic Acid Ther."},{"issue":"24","key":"10.1016\/j.matbio.2017.11.009_bb0415","doi-asserted-by":"crossref","first-page":"4914","DOI":"10.1093\/hmg\/ddt341","article-title":"Highly efficient in vivo delivery of PMO into regenerating myotubes and rescue in laminin-alpha2 chain-null congenital muscular dystrophy mice","volume":"22","author":"Aoki","year":"2013","journal-title":"Hum. Mol. Genet."},{"issue":"8","key":"10.1016\/j.matbio.2017.11.009_bb0420","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1038\/nm.4367","article-title":"Correction of a splicing defect in a mouse model of congenital muscular dystrophy type 1A using a homology-directed-repair-independent mechanism","volume":"23","author":"Kemaladewi","year":"2017","journal-title":"Nat. Med."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0425","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1152\/physrev.00033.2014","article-title":"Mechanisms regulating neuromuscular junction development and function and causes of muscle wasting","volume":"95","author":"Tintignac","year":"2015","journal-title":"Physiol. Rev."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0430","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/S0960-8966(03)00036-1","article-title":"Expression of mouse agrin in normal, denervated and dystrophic muscle","volume":"13","author":"Eusebio","year":"2003","journal-title":"Neuromuscul. Disord."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0435","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1083\/jcb.137.3.671","article-title":"Agrin binds to the nerve-muscle basal lamina via laminin","volume":"137","author":"Denzer","year":"1997","journal-title":"J. Cell Biol."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0440","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1093\/emboj\/17.2.335","article-title":"Electron microscopic structure of agrin and mapping of its binding site in laminin-1","volume":"17","author":"Denzer","year":"1998","journal-title":"EMBO J."},{"issue":"4","key":"10.1016\/j.matbio.2017.11.009_bb0445","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1016\/S0896-6273(00)80096-3","article-title":"Alternative splicing of agrin alters its binding to heparin, dystroglycan, and the putative agrin receptor","volume":"16","author":"Gesemann","year":"1996","journal-title":"Neuron"},{"issue":"8","key":"10.1016\/j.matbio.2017.11.009_bb0450","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1096\/fj.04-3376com","article-title":"Overexpression of mini-agrin in skeletal muscle increases muscle integrity and regenerative capacity in laminin-{alpha}2-deficient mice","volume":"19","author":"Bentzinger","year":"2005","journal-title":"FASEB J."},{"issue":"7","key":"10.1016\/j.matbio.2017.11.009_bb0455","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1083\/jcb.200611152","article-title":"Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stages","volume":"176","author":"Meinen","year":"2007","journal-title":"J. Cell Biol."},{"issue":"13","key":"10.1016\/j.matbio.2017.11.009_bb0460","doi-asserted-by":"crossref","first-page":"8984","DOI":"10.1074\/jbc.M809719200","article-title":"Scaffold-forming and adhesive contributions of synthetic laminin-binding proteins to basement membrane assembly","volume":"284","author":"McKee","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.matbio.2017.11.009_bb0465","doi-asserted-by":"crossref","first-page":"201","DOI":"10.3389\/fmicb.2011.00201","article-title":"Immunity and AAV-mediated gene therapy for muscular dystrophies in large animal models and human trials","volume":"2","author":"Wang","year":"2011","journal-title":"Front. Microbiol."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0470","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1517\/17425247.2014.871258","article-title":"The potential of adeno-associated viral vectors for gene delivery to muscle tissue","volume":"11","author":"Wang","year":"2014","journal-title":"Expert Opin. Drug Deliv."},{"issue":"2","key":"10.1016\/j.matbio.2017.11.009_bb0475","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.neulet.2012.04.078","article-title":"Gene therapy for muscular dystrophy: lessons learned and path forward","volume":"527","author":"Mendell","year":"2012","journal-title":"Neurosci. Lett."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0480","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0039233","article-title":"Homologous recombination mediates functional recovery of dysferlin deficiency following AAV5 gene transfer","volume":"7","author":"Grose","year":"2012","journal-title":"PLoS One"},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0485","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1038\/mt.2010.205","article-title":"Gene therapy of mdx mice with large truncated dystrophins generated by recombination using rAAV6","volume":"19","author":"Odom","year":"2011","journal-title":"Mol. Ther."},{"issue":"34","key":"10.1016\/j.matbio.2017.11.009_bb0490","doi-asserted-by":"crossref","first-page":"11999","DOI":"10.1073\/pnas.0502137102","article-title":"Amelioration of laminin-{alpha}2-deficient congenital muscular dystrophy by somatic gene transfer of miniagrin","volume":"102","author":"Qiao","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"3","key":"10.1016\/j.matbio.2017.11.009_bb0495","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1124\/jpet.109.160754","article-title":"Omigapil ameliorates the pathology of muscle dystrophy caused by laminin-alpha2 deficiency","volume":"331","author":"Erb","year":"2009","journal-title":"J. Pharmacol. Exp. Ther."},{"issue":"6","key":"10.1016\/j.matbio.2017.11.009_bb0500","doi-asserted-by":"crossref","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":"Yu","year":"2013","journal-title":"PLoS One"},{"issue":"21","key":"10.1016\/j.matbio.2017.11.009_bb0505","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."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0510","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."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0515","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"},{"issue":"1\u20132","key":"10.1016\/j.matbio.2017.11.009_bb0520","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/S0165-5728(02)00104-2","article-title":"Complement 3 deficiency and oral prednisolone improve strength and prolong survival of laminin alpha2-deficient mice","volume":"127","author":"Connolly","year":"2002","journal-title":"J. Neuroimmunol."},{"issue":"5","key":"10.1016\/j.matbio.2017.11.009_bb0525","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1016\/j.ajpath.2014.01.019","article-title":"Bortezomib partially improves laminin alpha2 chain-deficient muscular dystrophy","volume":"184","author":"Korner","year":"2014","journal-title":"Am. J. Pathol."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0530","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0146471","article-title":"Bortezomib does not reduce muscular dystrophy in the dy2J\/dy2J mouse model of laminin alpha2 chain-deficient muscular dystrophy","volume":"11","author":"Korner","year":"2016","journal-title":"PLoS One"},{"issue":"3508","key":"10.1016\/j.matbio.2017.11.009_bb0535","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1126\/science.135.3508.1061","article-title":"New genetically homogeneous background for dystrophic mice and their normal counterparts","volume":"135","author":"Russell","year":"1962","journal-title":"Science"},{"key":"10.1016\/j.matbio.2017.11.009_bb0540","doi-asserted-by":"crossref","first-page":"5572","DOI":"10.1073\/pnas.91.12.5572","article-title":"Defective muscle basement membrane and lack of M-laminin in the dystrophic dy\/dy mouse","volume":"91","author":"Xu","year":"1994","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.matbio.2017.11.009_bb0545","doi-asserted-by":"crossref","first-page":"13729","DOI":"10.1016\/S0021-9258(17)36704-2","article-title":"Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus","volume":"269","author":"Sunada","year":"1994","journal-title":"J. Biol. Chem."},{"issue":"19","key":"10.1016\/j.matbio.2017.11.009_bb0550","doi-asserted-by":"crossref","first-page":"7991","DOI":"10.1073\/pnas.0811599106","article-title":"Laminin-111 protein therapy prevents muscle disease in the mdx mouse model for Duchenne muscular dystrophy","volume":"106","author":"Rooney","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"10","key":"10.1016\/j.matbio.2017.11.009_bb0555","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1016\/S0960-8966(02)00180-3","article-title":"High dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy","volume":"12","author":"Connolly","year":"2002","journal-title":"Neuromuscul. Disord."},{"issue":"1","key":"10.1016\/j.matbio.2017.11.009_bb0560","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1002\/ana.21523","article-title":"Pathology is alleviated by doxycycline in a laminin-alpha2-null model of congenital muscular dystrophy","volume":"65","author":"Girgenrath","year":"2009","journal-title":"Ann. Neurol."}],"container-title":["Matrix Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0945053X17303335?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0945053X17303335?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T19:00:50Z","timestamp":1760554850000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0945053X17303335"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10]]},"references-count":112,"alternative-id":["S0945053X17303335"],"URL":"https:\/\/doi.org\/10.1016\/j.matbio.2017.11.009","relation":{},"ISSN":["0945-053X"],"issn-type":[{"value":"0945-053X","type":"print"}],"subject":[],"published":{"date-parts":[[2018,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies","name":"articletitle","label":"Article Title"},{"value":"Matrix Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.matbio.2017.11.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}