{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:49:06Z","timestamp":1792788546185,"version":"4.5.0"},"reference-count":51,"publisher":"Public Library of Science (PLoS)","issue":"9","license":[{"start":{"date-parts":[[2016,9,14]],"date-time":"2016-09-14T00:00:00Z","timestamp":1473811200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"LGMD2iFund"},{"name":"Carolinas Muscular Dystrophy Research Endowment"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0161984","type":"journal-article","created":{"date-parts":[[2016,9,14]],"date-time":"2016-09-14T13:33:25Z","timestamp":1473860005000},"page":"e0161984","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":20,"title":["Trendelenburg-Like Gait, Instability and Altered Step Patterns in a Mouse Model for Limb Girdle Muscular Dystrophy 2i"],"prefix":"10.1371","volume":"11","author":[{"given":"Joseph W.","family":"Maricelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi L.","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David C.","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Buel D.","family":"Rodgers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2016,9,14]]},"reference":[{"issue":"7","key":"ref1","doi-asserted-by":"crossref","first-page":"560","DOI":"10.3928\/0090-4481-20050701-13","article-title":"Congenital muscular dystrophy","volume":"34","author":"E Mercuri","year":"2005","journal-title":"Pediatric annals"},{"issue":"1","key":"ref2","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.pediatrneurol.2011.08.014","article-title":"Childhood onset of limb-girdle muscular dystrophy","volume":"46","author":"XQ Rosales","year":"2012","journal-title":"Pediatric neurology"},{"issue":"1","key":"ref3","first-page":"1","article-title":"Genetic basis of limb-girdle muscular dystrophies: the 2014 update","volume":"33","author":"V Nigro","year":"2014","journal-title":"Acta myologica: myopathies and cardiomyopathies: official journal of the Mediterranean Society of Myology \/ edited by the Gaetano Conte Academy for the study of striated muscle diseases"},{"issue":"6","key":"ref4","first-page":"474","article-title":"Dystroglycanopathy muscles lacking functional glycosylation of alpha-dystroglycan retain regeneration capacity. Neuromuscular disorders","volume":"25","author":"H Awano","year":"2015","journal-title":"NMD"},{"key":"ref5","first-page":"1","article-title":"Use of Whole-Exome Sequencing for Diagnosis of Limb-Girdle Muscular Dystrophy: Outcomes and Lessons Learned","author":"R Ghaoui","year":"2015","journal-title":"JAMA neurology"},{"issue":"3","key":"ref6","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.gde.2011.02.001","article-title":"Dystroglycanopathies: coming into focus","volume":"21","author":"C Godfrey","year":"2011","journal-title":"Curr Opin Genet Dev"},{"issue":"20","key":"ref7","doi-asserted-by":"crossref","first-page":"3995","DOI":"10.1093\/hmg\/ddq314","article-title":"Fukutin-related protein is essential for mouse muscle, brain and eye development and mutation recapitulates the wide clinical spectrums of dystroglycanopathies","volume":"19","author":"YM Chan","year":"2010","journal-title":"Hum Mol Genet"},{"issue":"8","key":"ref8","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1007\/s00439-013-1302-7","article-title":"Mouse models of fukutin-related protein mutations show a wide range of disease phenotypes","volume":"132","author":"A Blaeser","year":"2013","journal-title":"Human genetics"},{"issue":"6335","key":"ref9","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1038\/352536a0","article-title":"The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy","volume":"352","author":"HH Stedman","year":"1991","journal-title":"Nature"},{"issue":"8\u20139","key":"ref10","first-page":"491","article-title":"Evolution of the mdx mouse cardiomyopathy: physiological and morphological findings. Neuromuscular disorders","volume":"14","author":"JG Quinlan","year":"2004","journal-title":"NMD"},{"issue":"0","key":"ref11","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/j.ijcard.2014.12.090","article-title":"Progression of cardiac involvement in patients with limb-girdle type 2 and Becker muscular dystrophies: A 9-year follow-up study","volume":"182","author":"H Petri","year":"2015","journal-title":"International Journal of Cardiology"},{"issue":"5","key":"ref12","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1002\/mus.21432","article-title":"Cardiac pathology exceeds skeletal muscle pathology in two cases of limb-girdle muscular dystrophy type 2I","volume":"40","author":"M Margeta","year":"2009","journal-title":"Muscle Nerve"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.neuroscience.2008.08.041","article-title":"Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington&apos;s disease mice","volume":"157","author":"M Hickey","year":"2008","journal-title":"Neuroscience"},{"issue":"4","key":"ref14","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1002\/ana.10559","article-title":"Phenotypic spectrum associated with mutations in the fukutin-related protein gene","volume":"53","author":"E Mercuri","year":"2003","journal-title":"Ann Neurol"},{"issue":"12","key":"ref15","first-page":"1137","article-title":"A New Mouse Model of Limb-Girdle Muscular Dystrophy Type 2I Homozygous for the Common L276I Mutation Mimicking the Mild Phenotype in Humans","volume":"74","author":"TO Krag","year":"2015","journal-title":"Journal of neuropathology and experimental neurology"},{"issue":"10","key":"ref16","doi-asserted-by":"crossref","first-page":"1832","DOI":"10.1038\/mt.2013.156","article-title":"Adeno-associated virus 9 mediated FKRP gene therapy restores functional glycosylation of alpha-dystroglycan and improves muscle functions","volume":"21","author":"L Xu","year":"2013","journal-title":"Mol Ther"},{"issue":"18","key":"ref17","first-page":"262","article-title":"Muscle pathology, limb strength, walking gait, respiratory function and neurological impairment establish disease progression in the p.N155K canine model of X-linked myotubular myopathy","volume":"3","author":"MA Goddard","year":"2015","journal-title":"Annals of translational medicine"},{"issue":"Suppl 1","key":"ref18","doi-asserted-by":"crossref","first-page":"2042","DOI":"10.1080\/10255842.2015.1069615","article-title":"Changes in muscle activation during gait of children with duchenne muscular dystrophy","volume":"18","author":"J Ropars","year":"2015","journal-title":"Computer methods in biomechanics and biomedical engineering"},{"issue":"3","key":"ref19","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.gaitpost.2015.06.005","article-title":"Dynamic stability during level walking and obstacle crossing in persons with facioscapulohumeral muscular dystrophy","volume":"42","author":"NH Rijken","year":"2015","journal-title":"Gait & posture"},{"issue":"5","key":"ref20","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1002\/brb3.250","article-title":"Granulocyte colony-stimulating factor (G-CSF) positive effects on muscle fiber degeneration and gait recovery after nerve lesion in MDX mice","volume":"4","author":"GF Sim\u00f5es","year":"2014","journal-title":"Brain and Behavior"},{"issue":"7","key":"ref21","first-page":"341","article-title":"Analysis of gait and movement of upper limbs in muscular dystrophies","volume":"20","author":"Izco F Moreno","year":"2005","journal-title":"Neurologia (Barcelona, Spain)"},{"key":"ref22","article-title":"The 6 minute walk test and performance of upper limb in ambulant duchenne muscular dystrophy boys","volume":"6","author":"M Pane","year":"2014","journal-title":"PLoS currents"},{"issue":"4","key":"ref23","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1002\/mus.21544","article-title":"The 6-minute walk test as a new outcome measure in Duchenne muscular dystrophy","volume":"41","author":"CM McDonald","year":"2010","journal-title":"Muscle & nerve"},{"key":"ref24","article-title":"GeneReviews(R)","author":"E Pegoraro","year":"1993"},{"key":"ref25","article-title":"Genetic and Early Clinical Manifestations of Females Heterozygous for Duchenne\/Becker Muscular Dystrophy","author":"R Papa","year":"2015","journal-title":"Pediatric neurology"},{"issue":"6","key":"ref26","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1016\/S0031-3955(16)36146-6","article-title":"Limp","volume":"33","author":"RN Hensinger","year":"1986","journal-title":"Pediatric clinics of North America"},{"issue":"2","key":"ref27","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/S0883-5403(05)80013-0","article-title":"Significance of the Trendelenburg test in total hip arthroplasty. Influence of lateral approaches","volume":"11","author":"VS Pai","year":"1996","journal-title":"The Journal of arthroplasty"},{"issue":"1","key":"ref28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jneumeth.2008.06.017","article-title":"Using the CatWalk method to assess weight-bearing and pain behaviour in walking rats with ankle joint monoarthritis induced by carrageenan: Effects of morphine and rofecoxib","volume":"174","author":"K\u00c4 M\u00f6ller","year":"2008","journal-title":"Journal of Neuroscience Methods"},{"issue":"1","key":"ref29","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1186\/s12915-015-0154-0","article-title":"Quantification of gait parameters in freely walking rodents","volume":"13","author":"C Mendes","year":"2015","journal-title":"BMC Biology"},{"issue":"11","key":"ref30","first-page":"1426","article-title":"Phenotypic Characterization of Speed-Associated Gait Changes in Mice Reveals Modular Organization of Locomotor Networks. Current biology","volume":"25","author":"C Bellardita","year":"2015","journal-title":"CB"},{"issue":"6","key":"ref31","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1152\/japplphysiol.01014.2014","article-title":"Identification of mouse gaits using a novel force-sensing exercise wheel","volume":"119","author":"BJH Smith","year":"2015","journal-title":"Journal of Applied Physiology"},{"issue":"12","key":"ref32","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1590\/0004-282X20150168","article-title":"Limb-girdle muscular dystrophy type 2A in Brazilian children","volume":"73","author":"MA Albuquerque","year":"2015","journal-title":"Arquivos de neuro-psiquiatria"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1007\/978-94-011-6009-4_5","article-title":"Walking, running and jumping","author":"R Alexander","year":"1982","journal-title":"Locomotion of animals"},{"issue":"2","key":"ref34","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.nmd.2014.11.004","article-title":"Muscle force, balance and falls in muscular impaired individuals with myotonic dystrophy type 1: A five-year prospective cohort study","volume":"25","author":"E Hammar\u00e9n","year":"2015","journal-title":"Neuromuscular Disorders"},{"issue":"3","key":"ref35","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1002\/mus.23902","article-title":"The 6-minute walk test and other endpoints in Duchenne muscular dystrophy: longitudinal natural history observations over 48 weeks from a multicenter study","volume":"48","author":"CM McDonald","year":"2013","journal-title":"Muscle & nerve"},{"issue":"5","key":"ref36","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1111\/j.0021-8782.2004.00289.x","article-title":"Bipedal animals, and their differences from humans","volume":"204","author":"RM Alexander","year":"2004","journal-title":"Journal of Anatomy"},{"issue":"2","key":"ref37","doi-asserted-by":"crossref","first-page":"89","DOI":"10.5435\/00124635-200103000-00003","article-title":"The limping child: evaluation and diagnosis","volume":"9","author":"JM Flynn","year":"2001","journal-title":"The Journal of the American Academy of Orthopaedic Surgeons"},{"issue":"5","key":"ref38","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1007\/s00415-005-0684-4","article-title":"Diagnostic value of muscle MRI in differentiating LGMD2I from other LGMDs","volume":"252","author":"D Fischer","year":"2005","journal-title":"Journal of neurology"},{"issue":"3","key":"ref39","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1302\/0301-620X.79B3.7495","article-title":"Can Trendelenburg&apos;s sign be positive if the hip is normal?","volume":"79","author":"PN Vasudevan","year":"1997","journal-title":"The Journal of bone and joint surgery British volume"},{"issue":"3","key":"ref40","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1302\/0301-620X.68B3.3733820","article-title":"Delay in diagnosing Duchenne muscular dystrophy in orthopaedic clinics","volume":"68","author":"L Read","year":"1986","journal-title":"The Journal of bone and joint surgery British volume"},{"issue":"1","key":"ref41","first-page":"65","article-title":"Delayed diagnosis of Duchenne muscular dystrophy in Chile","volume":"127","author":"Avaria M de los Angeles","year":"1999","journal-title":"Revista medica de Chile"},{"key":"ref42","article-title":"Genetic and Early Clinical Manifestations of Females Heterozygous for Duchenne\/Becker Muscular Dystrophy","author":"R Papa","journal-title":"Pediatric neurology"},{"key":"ref43","doi-asserted-by":"crossref","unstructured":"Makino Y, Tsujiuchi N, Ito A, Koizumi T, Nakamura S, Matsuda Y, Tsuchiya Y, Hayashi Y. Quantitative evaluation of unrestrained human gait on change in walking velocity. Conference proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society IEEE Engineering in Medicine and Biology Society Annual Conference. 2014;2014:2521\u20134. Epub 2015\/01\/09. <comment>doi: <ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/dx.doi.org\/10.1109\/embc.2014.6944135\" xlink:type=\"simple\">10.1109\/embc.2014.6944135<\/ext-link><\/comment> 25570503.","DOI":"10.1109\/EMBC.2014.6944135"},{"key":"ref44","doi-asserted-by":"crossref","unstructured":"Adachi W, Tsujiuchi N, Koizumi T, Aikawa M, Shiojima K, Tsuchiya Y, Inoue Y. Development of walking analysis system consisting of mobile force plate and motion sensor. Conference proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society IEEE Engineering in Medicine and Biology Society Annual Conference. 2011;2011:4022\u20135. Epub 2012\/01\/19. <comment>doi: <ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/dx.doi.org\/10.1109\/iembs.2011.6090999\" xlink:type=\"simple\">10.1109\/iembs.2011.6090999<\/ext-link><\/comment> 22255222.","DOI":"10.1109\/IEMBS.2011.6090999"},{"issue":"48","key":"ref45","first-page":"e2138","article-title":"Kinematics and Ground Reaction Force Determination: A Demonstration Quantifying Locomotor Abilities of Young Adult, Middle-aged, and Geriatric Rats","author":"AA Webb","year":"2011","journal-title":"Kinematics and Ground Reaction Force Determination: A Demonstration Quantifying Locomotor Abilities of Young Adult, Middle-aged, and Geriatric Rats"},{"issue":"2","key":"ref46","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.pediatrneurol.2014.09.014","article-title":"Composite biomarkers for assessing Duchenne muscular dystrophy: an initial assessment","volume":"52","author":"I Shklyar","year":"2015","journal-title":"Pediatric neurology"},{"issue":"3","key":"ref47","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1002\/ana.21732","article-title":"Limb-girdle muscular dystrophy type 2D gene therapy restores alpha-sarcoglycan and associated proteins","volume":"66","author":"JR Mendell","year":"2009","journal-title":"Annals of neurology"},{"issue":"2","key":"ref48","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":"JR Mendell","year":"2012","journal-title":"Neurosci Lett"},{"issue":"1","key":"ref49","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":"GL Odom","year":"2011","journal-title":"Molecular therapy: the journal of the American Society of Gene Therapy"},{"issue":"6","key":"ref50","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1038\/mt.2008.76","article-title":"Analysis of AAV serotypes 1\u20139 mediated gene expression and tropism in mice after systemic injection","volume":"16","author":"C Zincarelli","year":"2008","journal-title":"Mol Ther"},{"issue":"25","key":"ref51","doi-asserted-by":"crossref","first-page":"13714","DOI":"10.1073\/pnas.240335297","article-title":"Adeno-associated virus vector carrying human minidystrophin genes effectively ameliorates muscular dystrophy in mdx mouse model","volume":"97","author":"B Wang","year":"2000","journal-title":"Proc Natl Acad Sci U S A"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0161984","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,9]],"date-time":"2020-05-09T07:31:57Z","timestamp":1589009517000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0161984"}},"subtitle":[],"editor":[{"given":"Diego","family":"Fraidenraich","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2016,9,14]]},"references-count":51,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2016,9,14]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0161984","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,14]]}}}