{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T20:50:08Z","timestamp":1784235008424,"version":"3.55.0"},"reference-count":55,"publisher":"Public Library of Science (PLoS)","issue":"4","license":[{"start":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T00:00:00Z","timestamp":1554854400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["418743"],"award-info":[{"award-number":["418743"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0214908","type":"journal-article","created":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T18:07:27Z","timestamp":1554919647000},"page":"e0214908","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":20,"title":["Dysferlin-deficiency has greater impact on function of slow muscles, compared with fast, in aged BLAJ mice"],"prefix":"10.1371","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9472-3450","authenticated-orcid":true,"given":"Erin M.","family":"Lloyd","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongyang","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn M.","family":"Murphy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miranda D.","family":"Grounds","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gavin J.","family":"Pinniger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2019,4,10]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/B978-0-08-045031-5.00007-4","article-title":"Dysferlinopathies","volume":"101","author":"AA Amato","year":"2011","journal-title":"Handb Clin Neurol"},{"issue":"1","key":"ref2","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1038\/1682","article-title":"Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy","volume":"20","author":"J Liu","year":"1998","journal-title":"Nat Genet"},{"issue":"4","key":"ref3","first-page":"e89","article-title":"The Clinical Outcome Study for dysferlinopathy: An international multicenter study. Neurology","volume":"2","author":"E Harris","year":"2016","journal-title":"Genetics"},{"issue":"2","key":"ref4","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1111\/j.1600-0854.2011.01267.x","article-title":"Ferlins: Regulators of vesicle fusion for auditory neurotransmission, receptor trafficking and membrane repair","volume":"13","author":"A Lek","year":"2012","journal-title":"Traffic"},{"issue":"6","key":"ref5","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1016\/j.ajpath.2014.02.005","article-title":"Lipid accumulation in dysferlin-deficient muscles","volume":"184","author":"MD Grounds","year":"2014","journal-title":"Am J Pathol"},{"issue":"2","key":"ref6","first-page":"134","article-title":"Intracellular localization of dysferlin and its association with the dihydropyridine receptor","volume":"24","author":"BN Ampong","year":"2005","journal-title":"Acta Myol"},{"issue":"11","key":"ref7","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1369\/0022155411423274","article-title":"Unmasking potential intracellular roles for dysferlin through improved immunolabeling methods","volume":"59","author":"JA Roche","year":"2011","journal-title":"J Histochem Cytochem"},{"issue":"2","key":"ref8","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1002\/mus.21166","article-title":"Dysferlin associates with the developing T-tubule system in rodent and human skeletal muscle","volume":"41","author":"L Klinge","year":"2010","journal-title":"Muscle Nerve"},{"issue":"8","key":"ref9","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1016\/j.biocel.2013.06.007","article-title":"Dysferlin interacts with calsequestrin-1, myomesin-2 and dynein in human skeletal muscle","volume":"45","author":"B Flix","year":"2013","journal-title":"Int J Biochem Cell Biol"},{"issue":"3","key":"ref10","first-page":"119","article-title":"Muscle fatigue, nNOS and muscle fiber atrophy in limb girdle muscular dystrophy","volume":"33","author":"C Angelini","year":"2014","journal-title":"Acta Myol"},{"issue":"5","key":"ref11","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1002\/mus.25367","article-title":"Progress and challenges in diagnosis of dysferlinopathy","volume":"54","author":"M Fanin","year":"2016","journal-title":"Muscle Nerve"},{"issue":"2","key":"ref12","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1136\/jcp.2004.018978","article-title":"Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism","volume":"58","author":"G Cenacchi","year":"2005","journal-title":"J Clin Pathol"},{"issue":"3","key":"ref13","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1002\/mus.24167","article-title":"Muscle atrophy, ubiquitin\u2013proteasome, and autophagic pathways in dysferlinopathy","volume":"50","author":"M Fanin","year":"2014","journal-title":"Muscle Nerve"},{"issue":"17","key":"ref14","doi-asserted-by":"crossref","first-page":"4149","DOI":"10.1111\/febs.12142","article-title":"Oxidative stress and pathology in muscular dystrophies: focus on protein thiol oxidation and dysferlinopathies","volume":"280","author":"JR Terrill","year":"2013","journal-title":"FEBS J"},{"issue":"1","key":"ref15","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.lfs.2014.05.005","article-title":"Involvement of oxidative stress, Nuclear Factor kappa B and the Ubiquitin proteasomal pathway in dysferlinopathy","volume":"108","author":"D Rajakumar","year":"2014","journal-title":"Life Sci"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1136\/jnnp-2017-317488","article-title":"Muscle MRI in patients with dysferlinopathy: pattern recognition and implications for clinical trials","volume":"89","author":"J Diaz-Manera","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"1","key":"ref17","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1016\/j.ajpath.2013.09.009","article-title":"Dysferlin and myoferlin regulate transverse tubule formation and glycerol sensitivity","volume":"184","author":"AR Demonbreun","year":"2014","journal-title":"Am J Pathol"},{"issue":"Pt A","key":"ref18","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/j.expneurol.2016.06.026","article-title":"Dysferlin function in skeletal muscle: Possible pathological mechanisms and therapeutical targets in dysferlinopathies","volume":"283","author":"AM C\u00e1rdenas","year":"2016","journal-title":"Exp Neurol"},{"issue":"3","key":"ref19","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1097\/00001756-200103050-00039","article-title":"Cloning of the mouse dysferlin gene and genomic characterization of the SJL-Dysf mutation","volume":"12","author":"E Vafiadaki","year":"2001","journal-title":"Neuroreport"},{"issue":"5","key":"ref20","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.nmd.2013.02.004","article-title":"Muscular dystrophy in dysferlin-deficient mouse models","volume":"23","author":"MA Hornsey","year":"2013","journal-title":"Neuromuscul Disord"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"134031","DOI":"10.1155\/2012\/134031","article-title":"Distinct effects of contraction-induced injury in vivo on four different murine models of dysferlinopathy","volume":"2012","author":"JA Roche","year":"2012","journal-title":"J Biomed Biotechnol"},{"key":"ref22","article-title":"Mouse models of dysferlin deficiency","author":"Jain Foundation","year":"2015","journal-title":"Mouse models of dysferlin deficiency"},{"issue":"16","key":"ref23","doi-asserted-by":"crossref","first-page":"1579","DOI":"10.1097\/WNR.0b013e328311ca35","article-title":"Impaired recovery of dysferlin-null skeletal muscle after contraction-induced injury in vivo","volume":"19","author":"JA Roche","year":"2008","journal-title":"Neuroreport"},{"issue":"2","key":"ref24","doi-asserted-by":"crossref","first-page":"C298","DOI":"10.1152\/ajpcell.00122.2009","article-title":"Extensive mononuclear infiltration and myogenesis characterize recovery of dysferlin-null skeletal muscle from contraction-induced injuries","volume":"298","author":"JA Roche","year":"2010","journal-title":"Am J Physiol-Cell Ph"},{"issue":"1","key":"ref25","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1002\/mus.21645","article-title":"Diaphragm displays early and progressive functional deficits in dysferlin-deficient mice","volume":"42","author":"ER Barton","year":"2010","journal-title":"Muscle Nerve"},{"issue":"0","key":"ref26","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/j.jns.2015.06.042","article-title":"Inhibition of inflammation with celastrol fails to improve muscle function in dysferlin-deficient A\/J mice","volume":"356","author":"BC Dillingham","year":"2015","journal-title":"J Neurol Sci"},{"issue":"6","key":"ref27","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1016\/j.ajpath.2013.02.045","article-title":"Dual effects of exercise in dysferlinopathy","volume":"182","author":"O Biondi","year":"2013","journal-title":"Am J Pathol"},{"issue":"11","key":"ref28","doi-asserted-by":"crossref","first-page":"1979","DOI":"10.1093\/hmg\/ddx065","article-title":"Annexin A2 links poor myofiber repair with inflammation and adipogenic replacement of the injured muscle","volume":"26","author":"A Defour","year":"2017","journal-title":"Hum Mol Genet"},{"issue":"6","key":"ref29","doi-asserted-by":"crossref","first-page":"e13173","DOI":"10.14814\/phy2.13173","article-title":"Hip region muscular dystrophy and emergence of motor deficits in dysferlin-deficient Bla\/J mice","volume":"5","author":"N Nagy","year":"2017","journal-title":"Physiological Reports"},{"issue":"9","key":"ref30","doi-asserted-by":"crossref","first-page":"e12981","DOI":"10.1371\/journal.pone.0012981","article-title":"Characterization of dysferlin deficient SJL\/J mice to assess preclinical drug efficacy: fasudil exacerbates muscle disease phenotype","volume":"5","author":"S Rayavarapu","year":"2010","journal-title":"PLoS One"},{"issue":"11","key":"ref31","doi-asserted-by":"crossref","first-page":"1976","DOI":"10.1093\/hmg\/ddp121","article-title":"Attenuated muscle regeneration is a key factor in dysferlin-deficient muscular dystrophy","volume":"18","author":"YH Chiu","year":"2009","journal-title":"Hum Mol Genet"},{"issue":"12","key":"ref32","doi-asserted-by":"crossref","first-page":"4366","DOI":"10.1172\/JCI42390","article-title":"Genetic ablation of complement C3 attenuates muscle pathology in dysferlin-deficient mice","volume":"120","author":"R Han","year":"2010","journal-title":"J Clin Invest"},{"issue":"2","key":"ref33","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1194\/jlr.M079459","article-title":"Increased nonHDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B","volume":"59","author":"SL Sellers","year":"2018","journal-title":"J Lipid Res"},{"issue":"2","key":"ref34","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1111\/sms.12416","article-title":"Effects of loaded voluntary wheel exercise on performance and muscle hypertrophy in young and old male C57Bl\/6J mice","volume":"26","author":"Z Soffe","year":"2016","journal-title":"Scand J Med Sci Sports"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1113\/jphysiol.1988.sp017279","article-title":"Contractile properties of skeletal muscles from young, adult and aged mice","volume":"404","author":"SV Brooks","year":"1988","journal-title":"J Physiol"},{"issue":"2","key":"ref36","first-page":"184","article-title":"Density and composition of mammalian muscle","volume":"9","author":"J Mendez","year":"1960","journal-title":"Metabolism"},{"issue":"5\u20136","key":"ref37","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1007\/s10974-017-9484-6","article-title":"Changes in contractile and metabolic parameters of skeletal muscle as rats age from 3 to 12 months","volume":"38","author":"H Xu","year":"2017","journal-title":"J Muscle Res Cell Motil"},{"issue":"732","key":"ref38","first-page":"101","article-title":"Assessment of muscle mass and strength in mice","volume":"4","author":"A Bonetto","year":"2015","journal-title":"Bonekey Rep"},{"issue":"1","key":"ref39","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2008.03.008","article-title":"Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy","volume":"31","author":"MD Grounds","year":"2008","journal-title":"Neurobiol Dis"},{"key":"ref40","article-title":"Brain Edema XV","author":"C Zhang","year":"2013"},{"issue":"4","key":"ref41","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1002\/cphy.c170052","article-title":"Immunobiology of Inherited Muscular Dystrophies","volume":"8","author":"JG Tidball","year":"2018","journal-title":"Compr Physiol"},{"issue":"1","key":"ref42","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1212\/WNL.50.1.41","article-title":"Unexpected sarcolemmal complement membrane attack complex deposits on nonnecrotic muscle fibers in muscular dystrophies","volume":"50","author":"S Spuler","year":"1998","journal-title":"Neurology"},{"issue":"9","key":"ref43","doi-asserted-by":"crossref","first-page":"6219","DOI":"10.4049\/jimmunol.175.9.6219","article-title":"Increased susceptibility to complement attack due to down-regulation of decay-accelerating factor\/CD55 in dysferlin-deficient muscular dystrophy","volume":"175","author":"K Wenzel","year":"2005","journal-title":"J Immunol"},{"issue":"5","key":"ref44","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1016\/j.molimm.2009.10.035","article-title":"The membrane attack complex of complement drives the progression of atherosclerosis in apolipoprotein E knockout mice","volume":"47","author":"RD Lewis","year":"2010","journal-title":"Mol Immunol"},{"issue":"1","key":"ref45","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1744-313X.1993.tb00090.x","article-title":"Studies on the Structure of Complement-C3 and the Stability of C3 Derived Phagocytic Ligands C3b\/Ic3b in Sjl\/J and Balb\/C Mice","volume":"20","author":"DM Lynch","year":"1993","journal-title":"Eur J Immunogenet"},{"issue":"15","key":"ref46","doi-asserted-by":"crossref","first-page":"5191","DOI":"10.1113\/JP274515","article-title":"Coupling of excitation to Ca2+ release is modulated by dysferlin","volume":"595","author":"V Lukyanenko","year":"2017","journal-title":"J Physiol"},{"issue":"89","key":"ref47","first-page":"1","article-title":"Dysferlin at transverse tubules regulates Ca2+ homeostasis in skeletal muscle","volume":"5","author":"JP Kerr","year":"2014","journal-title":"Front Physiol"},{"issue":"4","key":"ref48","doi-asserted-by":"crossref","first-page":"1718","DOI":"10.1152\/jn.1999.81.4.1718","article-title":"Force-Frequency and Fatigue Properties of Motor Units in Muscles That Control Digits of the Human Hand","volume":"81","author":"AJ Fuglevand","year":"1999","journal-title":"J Neurophysiol"},{"issue":"3","key":"ref49","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1152\/jn.00108.2014","article-title":"Characterization of motor units in behaving adult mice shows a wide primary range","volume":"112","author":"LK Ritter","year":"2014","journal-title":"J Neurophysiol"},{"issue":"51","key":"ref50","doi-asserted-by":"crossref","first-page":"20831","DOI":"10.1073\/pnas.1307960110","article-title":"Dysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane","volume":"110","author":"JP Kerr","year":"2013","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"3","key":"ref51","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1152\/physrev.2000.80.3.1215","article-title":"Calcium Ion in Skeletal Muscle: Its Crucial Role for Muscle Function, Plasticity, and Disease","volume":"80","author":"MW Berchtold","year":"2000","journal-title":"Physiol Rev"},{"issue":"2\u20134","key":"ref52","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S0079-6107(00)00006-7","article-title":"Human skeletal muscle fibres: molecular and functional diversity","volume":"73","author":"R Bottinelli","year":"2000","journal-title":"Prog Biophys Mol Biol"},{"issue":"6","key":"ref53","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1046\/j.1365-2990.2002.00417.x","article-title":"Muscle pathology in dysferlin deficiency","volume":"28","author":"M Fanin","year":"2002","journal-title":"Neuropathol Appl Neurobiol"},{"issue":"23","key":"ref54","article-title":"Transcriptome-scale similarities between mouse and human skeletal muscles with normal and myopathic phenotypes","volume":"7","author":"AT Kho","year":"2006","journal-title":"BMC Musculoskel Disord"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1093\/brain\/109.1.31","article-title":"Autosomal Recessive Distal Muscular-Dystrophy as a New Type of Progressive Muscular-Dystrophy\u201417 Cases in 8 Families Including an Autopsied Case","volume":"109","author":"K Miyoshi","year":"1986","journal-title":"Brain"}],"updated-by":[{"DOI":"10.1371\/journal.pone.0286286","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2023,5,22]],"date-time":"2023-05-22T00:00:00Z","timestamp":1684713600000}}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0214908","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,9]],"date-time":"2020-05-09T02:13:12Z","timestamp":1588990392000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0214908"}},"subtitle":[],"editor":[{"given":"Atsushi","family":"Asakura","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2019,4,10]]},"references-count":55,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2019,4,10]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0214908","relation":{"correction":[{"id-type":"doi","id":"10.1371\/journal.pone.0286286","asserted-by":"object"}]},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,4,10]]}}}