{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T01:16:13Z","timestamp":1792718173116,"version":"4.5.0"},"reference-count":48,"publisher":"Public Library of Science (PLoS)","issue":"1","license":[{"start":{"date-parts":[[2016,1,11]],"date-time":"2016-01-11T00:00:00Z","timestamp":1452470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0147049","type":"journal-article","created":{"date-parts":[[2016,1,11]],"date-time":"2016-01-11T18:50:39Z","timestamp":1452538239000},"page":"e0147049","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":8,"title":["Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice"],"prefix":"10.1371","volume":"11","author":[{"given":"Steven J.","family":"Foltz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jill N.","family":"Modi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garrett A.","family":"Melick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marin I.","family":"Abousaud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junna","family":"Luan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa J.","family":"Fortunato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron M.","family":"Beedle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2016,1,11]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1016\/0092-8674(91)90035-W","article-title":"Membrane organization of the dystrophin-glycoprotein complex","volume":"66","author":"JM Ervasti","year":"1991","journal-title":"Cell"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1038\/355696a0","article-title":"Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix","volume":"355","author":"O Ibraghimov-Beskrovnaya","year":"1992","journal-title":"Nature"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1083\/jcb.112.1.135","article-title":"Dystrophin-glycoprotein complex is highly enriched in isolated skeletal muscle sarcolemma","volume":"112","author":"K Ohlendieck","year":"1991","journal-title":"J Cell Biol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1083\/jcb.122.4.809","article-title":"A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin","volume":"122","author":"JM Ervasti","year":"1993","journal-title":"J Cell Biol"},{"key":"ref5","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":"JF Talts","year":"1999","journal-title":"EMBO J"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/0092-8674(94)90045-0","article-title":"Dystrophin-associated proteins and synapse formation: is alpha-dystroglycan the agrin receptor?","volume":"77","author":"R Sealock","year":"1994","journal-title":"Cell"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/0092-8674(94)90052-3","article-title":"Dystroglycan-alpha, a dystrophin-associated glycoprotein, is a functional agrin receptor","volume":"77","author":"SH Gee","year":"1994","journal-title":"Cell"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"475","DOI":"10.3109\/15419069809005605","article-title":"The relationship between perlecan and dystroglycan and its implication in the formation of the neuromuscular junction","volume":"5","author":"HB Peng","year":"1998","journal-title":"Cell Adhes Commun"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"4792","DOI":"10.1016\/j.febslet.2005.07.059","article-title":"Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function","volume":"579","author":"M Kanagawa","year":"2005","journal-title":"FEBS Lett"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"27585","DOI":"10.1074\/jbc.M114.595413","article-title":"Dystroglycan binding to alpha-neurexin competes with neurexophilin-1 and neuroligin in the brain","volume":"289","author":"C Reissner","year":"2014","journal-title":"J Biol Chem"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/j.bbamem.2013.08.023","article-title":"Dystrophin complex functions as a scaffold for signalling proteins","volume":"1838","author":"B Constantin","year":"2014","journal-title":"Biochim Biophys Acta"},{"key":"ref12","article-title":"Spectrum of muscular dystrophies associated with sarcolemmal-protein genetic defects","author":"V Nigro","year":"2014","journal-title":"Biochim Biophys Acta"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"2392","DOI":"10.1002\/cbic.201300417","article-title":"Dissecting the molecular basis of the role of the O-mannosylation pathway in disease: alpha-dystroglycan and forms of muscular dystrophy","volume":"14","author":"D Live","year":"2013","journal-title":"Chembiochem"},{"key":"ref14","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":"R Han","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"3330","DOI":"10.1172\/JCI63004","article-title":"Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy","volume":"122","author":"AM Beedle","year":"2012","journal-title":"J Clin Invest"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"3003","DOI":"10.1093\/hmg\/ddt157","article-title":"Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration of its severe phenotype by limited gene expression","volume":"22","author":"M Kanagawa","year":"2013","journal-title":"Hum Mol Genet"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1038\/nature12605","article-title":"LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy","volume":"503","author":"MM Goddeeris","year":"2013","journal-title":"Nature"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"5781","DOI":"10.1093\/hmg\/ddu296","article-title":"POMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentations","volume":"23","author":"S Di Costanzo","year":"2014","journal-title":"Hum Mol Genet"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1152\/physrev.00031.2010","article-title":"Fiber types in mammalian skeletal muscles","volume":"91","author":"S Schiaffino","year":"2011","journal-title":"Physiol Rev"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1016\/0012-1606(84)90197-0","article-title":"Myosin isozyme transitions occurring during the postnatal development of the rat soleus muscle","volume":"102","author":"GS Butler-Browne","year":"1984","journal-title":"Dev Biol"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/B978-0-12-416022-4.00006-8","article-title":"Satellite cells: the architects of skeletal muscle","volume":"107","author":"NC Chang","year":"2014","journal-title":"Curr Top Dev Biol"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/S0960-8966(98)00114-X","article-title":"Correlation of muscle fiber type measurements with clinical and molecular genetic data in Duchenne muscular dystrophy","volume":"9","author":"JF Wang","year":"1999","journal-title":"Neuromuscul Disord"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/0960-8966(95)00016-X","article-title":"Skeletal muscle sarcoplasmic reticulum phenotype in myotonic dystrophy","volume":"6","author":"E Damiani","year":"1996","journal-title":"Neuromuscul Disord"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"2191","DOI":"10.1093\/hmg\/ddq098","article-title":"Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres","volume":"19","author":"C Trollet","year":"2010","journal-title":"Hum Mol Genet"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"R124","DOI":"10.1152\/ajpregu.00379.2013","article-title":"Altered Tnnt3 characterizes selective weakness of fast fibers in mice overexpressing FSHD region gene 1 (FRG1)","volume":"306","author":"V Sancisi","year":"2014","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"C1430","DOI":"10.1152\/ajpcell.00192.2010","article-title":"Soleus muscle in glycosylation-deficient muscular dystrophy is protected from contraction-induced injury","volume":"299","author":"JD Gumerson","year":"2010","journal-title":"Am J Physiol Cell Physiol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"16713","DOI":"10.1074\/jbc.C700061200","article-title":"Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex","volume":"282","author":"AM Beedle","year":"2007","journal-title":"J Biol Chem"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"e97567","DOI":"10.1371\/journal.pone.0097567","article-title":"Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue","volume":"9","author":"MJ Fortunato","year":"2014","journal-title":"PLoS One"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"e106721","DOI":"10.1371\/journal.pone.0106721","article-title":"Contribution of dysferlin deficiency to skeletal muscle pathology in asymptomatic and severe dystroglycanopathy models: generation of a new model for Fukuyama congenital muscular dystrophy","volume":"9","author":"M Kanagawa","year":"2014","journal-title":"PLoS One"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"3346","DOI":"10.1093\/hmg\/ddr240","article-title":"Sarcolemma instability during mechanical activity in Largemyd cardiac myocytes with loss of dystroglycan extracellular matrix receptor function","volume":"20","author":"Z Kabaeva","year":"2011","journal-title":"Hum Mol Genet"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/0012-1606(92)90221-2","article-title":"Temporal and quantitative analysis of myogenic regulatory and growth factor gene expression in the developing mouse embryo","volume":"151","author":"K Hannon","year":"1992","journal-title":"Dev Biol"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1242\/dev.111.4.1097","article-title":"Early expression of the myogenic regulatory gene, myf-5, in precursor cells of skeletal muscle in the mouse embryo","volume":"111","author":"MO Ott","year":"1991","journal-title":"Development"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1097\/NEN.0b013e31816b4acc","article-title":"Type 2 myotonic dystrophy can be predicted by the combination of type 2 muscle fiber central nucleation and scattered atrophy","volume":"67","author":"G Bassez","year":"2008","journal-title":"J Neuropathol Exp Neurol"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.nmd.2003.09.008","article-title":"Molecular phenotyping of the mouse ky mutant reveals UCP1 upregulation at the neuromuscular junctions of dystrophic soleus muscle","volume":"14","author":"G Blanco","year":"2004","journal-title":"Neuromuscul Disord"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"3478","DOI":"10.1093\/hmg\/ddr265","article-title":"Chronic AMPK activation evokes the slow, oxidative myogenic program and triggers beneficial adaptations in mdx mouse skeletal muscle","volume":"20","author":"V Ljubicic","year":"2011","journal-title":"Hum Mol Genet"},{"issue":"Suppl 3","key":"ref36","doi-asserted-by":"crossref","first-page":"S214","DOI":"10.1016\/j.nmd.2012.10.010","article-title":"Fatigue in muscular dystrophies","volume":"22","author":"C Angelini","year":"2012","journal-title":"Neuromuscul Disord"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1007\/s10974-010-9229-2","article-title":"Posttetanic potentiation in mdx muscle","volume":"31","author":"IC Smith","year":"2010","journal-title":"J Muscle Res Cell Motil"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1113\/jphysiol.1990.sp017916","article-title":"Physiological properties of skinned fibres from normal and dystrophic (Duchenne) human muscle activated by Ca2+ and Sr2+","volume":"420","author":"RH Fink","year":"1990","journal-title":"J Physiol"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/j.nmd.2013.10.005","article-title":"Characteristics of magnetic resonance imaging biomarkers in a natural history study of golden retriever muscular dystrophy","volume":"24","author":"Z Fan","year":"2014","journal-title":"Neuromuscul Disord"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1007\/s10974-006-9066-5","article-title":"Characteristics of muscle fibers reconstituted in the regeneration process of masseter muscle in an mdx mouse model of muscular dystrophy","volume":"27","author":"WH Lee","year":"2006","journal-title":"J Muscle Res Cell Motil"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1002\/mus.880050204","article-title":"Muscle histochemistry in congenital muscular dystrophy with central nervous system involvement","volume":"5","author":"I Nonaka","year":"1982","journal-title":"Muscle Nerve"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1590\/S0100-879X2002000600009","article-title":"Impaired regeneration of dystrophin-deficient muscle fibers is caused by exhaustion of myogenic cells","volume":"35","author":"MA Luz","year":"2002","journal-title":"Braz J Med Biol Res"},{"issue":"Pt 12","key":"ref43","doi-asserted-by":"crossref","first-page":"2299","DOI":"10.1242\/jcs.113.12.2299","article-title":"Evidence for a myogenic stem cell that is exhausted in dystrophic muscle","volume":"113","author":"L Heslop","year":"2000","journal-title":"J Cell Sci"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1093\/hmg\/ddl045","article-title":"Aberrant neuromuscular junctions and delayed terminal muscle fiber maturation in alpha-dystroglycanopathies","volume":"15","author":"M Taniguchi","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1002\/1097-0029(20000915)50:6<500::AID-JEMT7>3.0.CO;2-7","article-title":"Myosin isoforms, muscle fiber types, and transitions","volume":"50","author":"D Pette","year":"2000","journal-title":"Microsc Res Tech"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"12095","DOI":"10.1074\/jbc.274.17.12095","article-title":"The CACC box and myocyte enhancer factor-2 sites within the myosin light chain 2 slow promoter cooperate in regulating nerve-specific transcription in skeletal muscle","volume":"274","author":"K Esser","year":"1999","journal-title":"J Biol Chem"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1111\/j.1432-0436.1993.tb00642.x","article-title":"Satellite Cells from Slow Rat Muscle Express Slow Myosin under Appropriate Culture Conditions","volume":"53","author":"S Dusterhoft","year":"1993","journal-title":"Differentiation"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1046\/j.1432-0436.1996.6010039.x","article-title":"Phenotype of adult mouse muscle myoblasts reflects their fiber type of origin","volume":"60","author":"JD Rosenblatt","year":"1996","journal-title":"Differentiation"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0147049","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T10:31:13Z","timestamp":1654079473000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0147049"}},"subtitle":[],"editor":[{"given":"Renzhi","family":"Han","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2016,1,11]]},"references-count":48,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2016,1,11]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0147049","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,1,11]]}}}