{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T04:20:19Z","timestamp":1790828419152,"version":"4.1.0"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,4,4]],"date-time":"2021-04-04T00:00:00Z","timestamp":1617494400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Japan Agency for Medical AQ7 Research and Development","award":["19bm0804005-h0103"],"award-info":[{"award-number":["19bm0804005-h0103"]}]},{"name":"Japan Agency for Medical AQ7 Research and Development","award":["20lm0203086h0002"],"award-info":[{"award-number":["20lm0203086h0002"]}]},{"name":"Scientific Research","award":["19K075190001"],"award-info":[{"award-number":["19K075190001"]}]},{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Neurological and Psychiatric Disorders of the National Center of Neurology and Psychiatry","award":["28-6"],"award-info":[{"award-number":["28-6"]}]},{"name":"Neurological and Psychiatric Disorders of the National Center of Neurology and Psychiatry","award":["30-9"],"award-info":[{"award-number":["30-9"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,5,31]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscular weakness because of the loss of dystrophin. Extracellular Ca2+ flows into the cytoplasm through membrane tears in dystrophin-deficient myofibers, which leads to muscle contracture and necrosis. Sarco\/endoplasmic reticulum Ca2+-ATPase (SERCA) takes up cytosolic Ca2+ into the sarcoplasmic reticulum, but its activity is decreased in dystrophic muscle. Here, we show that an allosteric SERCA activator, CDN1163, ameliorates dystrophic phenotypes in dystrophin-deficient mdx mice. The administration of CDN1163 prevented exercise-induced muscular damage and restored mitochondrial function. In addition, treatment with CDN1163 for 7 weeks enhanced muscular strength and reduced muscular degeneration and fibrosis in mdx mice. Our findings provide preclinical proof-of-concept evidence that pharmacological activation of SERCA could be a promising therapeutic strategy for DMD. Moreover, CDN1163 improved muscular strength surprisingly in wild-type mice, which may pave the new way for the treatment of muscular dysfunction.<\/jats:p>","DOI":"10.1093\/hmg\/ddab100","type":"journal-article","created":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T11:39:25Z","timestamp":1617277165000},"page":"1006-1019","source":"Crossref","is-referenced-by-count":50,"title":["Pharmacological activation of SERCA ameliorates dystrophic phenotypes in dystrophin-deficient <i>mdx<\/i> mice"],"prefix":"10.1093","volume":"30","author":[{"given":"Ken'ichiro","family":"Nogami","sequence":"first","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"},{"name":"Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Maruyama","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"},{"name":"Department of Gene Regulation, Faculty of Pharmaceutical Sciences, Tokyo University of Science, Noda, Chiba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fusako","family":"Sakai-Takemura","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norio","family":"Motohashi","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Elhussieny","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"},{"name":"Department of Neurology, Faculty of Medicine, Minia University, Minia, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michihiro","family":"Imamura","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Miyashita","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Cellular Biology, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megumu","family":"Ogawa","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Research, National Institute of Neuroscience, Translational Medical Center, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoru","family":"Noguchi","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Research, National Institute of Neuroscience, Translational Medical Center, National Center of Neurology and Psychiatry, Tokyo, Japan"},{"name":"Department of Clinical Development, Translational Medical Center, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuki","family":"Tamura","sequence":"additional","affiliation":[{"name":"Graduate School of Health and Sport Science, Nippon Sport Science University, Tokyo, Japan"},{"name":"Research Institute for Sport Science, Nippon Sport Science University, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun-ichi","family":"Kira","sequence":"additional","affiliation":[{"name":"Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshitsugu","family":"Aoki","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shin'ichi","family":"Takeda","sequence":"additional","affiliation":[{"name":"National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Miyagoe-Suzuki","sequence":"additional","affiliation":[{"name":"Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,4,5]]},"reference":[{"key":"2021070819095474800_ref1","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/0092-8674(87)90579-4","article-title":"Dystrophin: the protein product of the Duchenne muscular dystrophy locus","volume":"51","author":"Hoffman","year":"1987","journal-title":"Cell"},{"key":"2021070819095474800_ref2","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/S1474-4422(18)30024-3","article-title":"Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management","volume":"17","author":"Birnkrant","year":"2018","journal-title":"Lancet Neurol."},{"key":"2021070819095474800_ref3","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1016\/j.nmd.2014.03.008","article-title":"A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy","volume":"24","author":"Mah","year":"2014","journal-title":"Neuromuscul. Disord."},{"key":"2021070819095474800_ref4","first-page":"121","article-title":"Improvement of survival in Duchenne Muscular Dystrophy: retrospective analysis of 835 patients","volume":"31","author":"Passamano","year":"2012","journal-title":"Acta. Myol."},{"key":"2021070819095474800_ref5","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1038\/333861a0","article-title":"Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne muscular dystrophy peptide","volume":"333","author":"Arahata","year":"1988","journal-title":"Nature"},{"key":"2021070819095474800_ref6","doi-asserted-by":"crossref","first-page":"798","DOI":"10.2183\/pjab.86.798","article-title":"Our trails and trials in the subsarcolemmal cytoskeleton network and muscular dystrophy researches in the dystrophin era","volume":"86","author":"Ozawa","year":"2010","journal-title":"Proc. Jpn. Acad. Ser B. Phys. Biol. Sci."},{"key":"2021070819095474800_ref7","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1002\/mus.880170103","article-title":"Dystrophin-glycoprotein complex: its role in the molecular pathogenesis of muscular dystrophies","volume":"17","author":"Matsumura","year":"1994","journal-title":"Muscle Nerve"},{"key":"2021070819095474800_ref8","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1083\/jcb.200301101","article-title":"A novel mechanism of myocyte degeneration involving the Ca2+-permeable growth factor-regulated channel","volume":"161","author":"Iwata","year":"2003","journal-title":"J. Cell Biol."},{"key":"2021070819095474800_ref9","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1097\/WCO.0b013e328364fbaf","article-title":"Pharmacological prospects in the treatment of Duchenne muscular dystrophy","volume":"26","author":"Ruegg","year":"2013","journal-title":"Curr. Opin. Neurol."},{"key":"2021070819095474800_ref10","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0049862","article-title":"Orai1 mediates exacerbated Ca2+ entry in dystrophic skeletal muscle","volume":"7","author":"Zhao","year":"2012","journal-title":"PLoS One"},{"key":"2021070819095474800_ref11","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1152\/physrev.00007.2015","article-title":"Absence of dystrophin disrupts skeletal muscle signaling: roles of Ca2+, reactive oxygen species, and nitric oxide in the development of muscular dystrophy","volume":"96","author":"Allen","year":"2016","journal-title":"Physiol. Rev."},{"key":"2021070819095474800_ref12","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1038\/nm.1916","article-title":"Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle","volume":"15","author":"Bellinger","year":"2009","journal-title":"Nat. Med."},{"key":"2021070819095474800_ref13","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s10974-013-9350-0","article-title":"Increased sarcolipin expression and decreased sarco(endo)plasmic reticulum Ca2+ uptake in skeletal muscles of mouse models of Duchenne muscular dystrophy","volume":"34","author":"Schneider","year":"2013","journal-title":"J. Muscle Res. Cell Motil."},{"key":"2021070819095474800_ref14","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1002\/mus.20745","article-title":"SERCA pump isoforms: their role in calcium transport and disease","volume":"35","author":"Periasamy","year":"2007","journal-title":"Muscle Nerve"},{"key":"2021070819095474800_ref15","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1172\/JCI43844","article-title":"Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle","volume":"121","author":"Goonasekera","year":"2011","journal-title":"J. Clin. Invest."},{"key":"2021070819095474800_ref16","doi-asserted-by":"crossref","first-page":"1735","DOI":"10.1089\/hum.2010.077","article-title":"Overexpression of SERCA1a in the mdx diaphragm reduces susceptibility to contraction-induced damage","volume":"21","author":"Morine","year":"2010","journal-title":"Hum. Gene Ther."},{"key":"2021070819095474800_ref17","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1186\/1479-5876-9-132","article-title":"SERCA2a gene transfer improves electrocardiographic performance in aged mdx mice","volume":"9","author":"Shin","year":"2011","journal-title":"J. Transl. Med."},{"key":"2021070819095474800_ref18","doi-asserted-by":"crossref","first-page":"C699","DOI":"10.1152\/ajpcell.00341.2014","article-title":"SERCA1 overexpression minimizes skeletal muscle damage in dystrophic mouse models","volume":"308","author":"M\u00e1zala","year":"2015","journal-title":"Am. J. Physiol. Cell. Physiol."},{"key":"2021070819095474800_ref19","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1038\/nature10980","article-title":"Hsp72 preserves muscle function and slows progression of severe muscular dystrophy","volume":"484","author":"Gehrig","year":"2012","journal-title":"Nature"},{"key":"2021070819095474800_ref20","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1177\/1087057112456878","article-title":"High-throughput FRET assay yields allosteric SERCA activators","volume":"18","author":"Cornea","year":"2013","journal-title":"J. Biomol. Screen."},{"key":"2021070819095474800_ref21","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1177\/1087057113510740","article-title":"Discovery of enzyme modulators via high-throughput time-resolved FRET in living cells","volume":"19","author":"Gruber","year":"2014","journal-title":"J. Biomol. Screen."},{"key":"2021070819095474800_ref22","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.bmc.2016.10.008","article-title":"A new target for Parkinson\u2019s disease: small molecule SERCA activator CDN1163 ameliorates dyskinesia in 6-OHDA-lesioned rats","volume":"25","author":"Dahl","year":"2017","journal-title":"Bioorg. Med. Chem."},{"key":"2021070819095474800_ref23","doi-asserted-by":"crossref","first-page":"1591","DOI":"10.1016\/j.bmcl.2018.03.052","article-title":"A new target for Alzheimer's disease: A small molecule SERCA activator is neuroprotective in vitro and improves memory and cognition in APP\/PS1 mice","volume":"28","author":"Krajnak","year":"2018","journal-title":"Bioorg. Med. Chem."},{"key":"2021070819095474800_ref24","doi-asserted-by":"crossref","first-page":"5185","DOI":"10.1074\/jbc.M115.705012","article-title":"Small Molecular Allosteric Activator of the Sarco\/Endoplasmic Reticulum Ca2+-ATPase (SERCA) Attenuates diabetes and metabolic disorders","volume":"291","author":"Kang","year":"2016","journal-title":"J. Biol. Chem."},{"key":"2021070819095474800_ref25","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.redox.2018.09.018","article-title":"Restoration of SERCA ATPase prevents oxidative stress-related muscle atrophy and weakness","volume":"20","author":"Qaisar","year":"2019","journal-title":"Redox Biol."},{"key":"2021070819095474800_ref26","doi-asserted-by":"crossref","first-page":"37","DOI":"10.3390\/ijms22010037","article-title":"Restoration of sarcoplasmic reticulum Ca2+ATPase (SERCA) activity prevents age-related muscle atrophy and weakness in mice","volume":"22","author":"Qaisar","year":"2020","journal-title":"Int. J. Mol. Sci."},{"key":"2021070819095474800_ref27","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13395-020-0221-2","article-title":"Mechanical factors tune the sensitivity of mdx muscle to eccentric strength loss and its protection by antioxidant and calcium modulators","volume":"10","author":"Lindsay","year":"2020","journal-title":"Skelet. Muscle"},{"key":"2021070819095474800_ref28","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/j.nmd.2011.11.007","article-title":"N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis","volume":"22","author":"Terrill","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"2021070819095474800_ref29","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1093\/jb\/118.5.959","article-title":"Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: evidence of apoptosis in dystrophin-deficient muscle","volume":"118","author":"Matsuda","year":"1995","journal-title":"J. Biochem."},{"key":"2021070819095474800_ref30","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1038\/nm1736","article-title":"Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy","volume":"14","author":"Millay","year":"2008","journal-title":"Nat. Med."},{"key":"2021070819095474800_ref31","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1038\/bjp.2008.285","article-title":"Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy","volume":"155","author":"Reutenauer","year":"2008","journal-title":"Br. J. Pharmacol."},{"key":"2021070819095474800_ref32","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/S0006-291X(03)00616-8","article-title":"Calcium signaling and apoptosis","volume":"304","author":"Hajn\u00f3czky","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"2021070819095474800_ref33","doi-asserted-by":"crossref","first-page":"H144","DOI":"10.1152\/ajpheart.00522.2010","article-title":"Stress-induced opening of the permeability transition pore in the dystrophin-deficient heart is attenuated by acute treatment with sildenafil","volume":"300","author":"Ascah","year":"2011","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"2021070819095474800_ref34","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1093\/hmg\/dds415","article-title":"Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibition","volume":"22","author":"Percival","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"2021070819095474800_ref35","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/nrm2125","article-title":"Matrix metalloproteinases and the regulation of tissue remodelling","volume":"8","author":"Page-McCaw","year":"2007","journal-title":"Nat. Rev. Mol. Cell. Biol."},{"key":"2021070819095474800_ref36","doi-asserted-by":"crossref","first-page":"2123","DOI":"10.1101\/gad.815400","article-title":"Matrix metalloproteinases: effectors of development and normal physiology","volume":"14","author":"Vu","year":"2000","journal-title":"Genes Dev."},{"key":"2021070819095474800_ref37","doi-asserted-by":"crossref","first-page":"2584","DOI":"10.1093\/hmg\/ddp191","article-title":"Matrix metalloproteinase-9 inhibition ameliorates pathogenesis and improves skeletal muscle regeneration in muscular dystrophy","volume":"18","author":"Li","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"2021070819095474800_ref38","doi-asserted-by":"crossref","first-page":"C296","DOI":"10.1152\/ajpcell.1987.253.2.C296","article-title":"Changes in intracellular ionized Ca concentration associated with muscle fiber type transformation","volume":"253","author":"Sreter","year":"1987","journal-title":"Am. J. Phys."},{"key":"2021070819095474800_ref39","doi-asserted-by":"crossref","first-page":"2499","DOI":"10.1101\/gad.12.16.2499","article-title":"A calcineurin-dependent transcriptional pathway controls skeletal muscle fiber type","volume":"12","author":"Chin","year":"1998","journal-title":"Genes Dev."},{"key":"2021070819095474800_ref40","doi-asserted-by":"crossref","first-page":"45323","DOI":"10.1074\/jbc.M208302200","article-title":"Intracellular calcium and myosin isoform transitions. Calcineurin and calcium-calmodulin kinase pathways regulate preferential activation of the IIa myosin heavy chain promoter","volume":"77","author":"Allen","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2021070819095474800_ref41","doi-asserted-by":"crossref","first-page":"2990","DOI":"10.1096\/fj.07-097600","article-title":"Modulation of skeletal muscle fiber type by mitogen-activated protein kinase signaling","volume":"22","author":"Shi","year":"2008","journal-title":"FASEB J."},{"key":"2021070819095474800_ref42","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.127356","article-title":"ERK1\/2 signaling induces skeletal muscle slow fiber-type switching and reduces muscular dystrophy disease severity","volume":"4","author":"Boyer","year":"2019","journal-title":"JCI. Insight"},{"key":"2021070819095474800_ref43","doi-asserted-by":"crossref","first-page":"7168","DOI":"10.1038\/s41598-017-07149-0","article-title":"Vestigial-like 2 contributes to normal muscle fiber type distribution in mice","volume":"7","author":"Honda","year":"2017","journal-title":"Sci. Rep."},{"key":"2021070819095474800_ref44","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1038\/cdd.2016.127","article-title":"Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy","volume":"24","author":"Vila","year":"2017","journal-title":"Cell Death Differ."},{"key":"2021070819095474800_ref45","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/0022-510X(94)00276-T","article-title":"mdx mice show progressive weakness and muscle deterioration with age","volume":"129","author":"Pastoret","year":"1995","journal-title":"J. Neurol. Sci."},{"key":"2021070819095474800_ref46","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1006\/dbio.1994.1103","article-title":"Myogenic cell lines derived from transgenic mice carrying a thermolabile T antigen: a model system for the derivation of tissue-specific and mutation-specific cell lines","volume":"162","author":"Morgan","year":"1994","journal-title":"Dev. Biol."},{"key":"2021070819095474800_ref47","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/nm.3019","article-title":"Activation of calcium signaling through Trpv1 by nNOS and peroxynitrite as a key trigger of skeletal muscle hypertrophy","volume":"19","author":"Ito","year":"2013","journal-title":"Nat. Med."},{"key":"2021070819095474800_ref48","article-title":"Isometric and eccentric force generation assessment of skeletal muscles isolated from murine models of muscular dystrophies","volume":"71","author":"Moorwood","year":"2013","journal-title":"J. Vis. Exp."},{"key":"2021070819095474800_ref49","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1111\/j.1469-7793.2001.00591.x","article-title":"Force and power output of fast and slow skeletal muscles from mdx mice 6-28 months old","volume":"535","author":"Lynch","year":"2001","journal-title":"J. Physiol."},{"key":"2021070819095474800_ref50","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1152\/physiolgenomics.90219.2008","article-title":"Muscle weakness correlates with muscle atrophy and precedes the development of inclusion body or rimmed vacuoles in the mouse model of DMRV\/hIBM","volume":"35","author":"Malicdan","year":"2008","journal-title":"Physiol. Genomics"},{"key":"2021070819095474800_ref51","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1073\/pnas.0608277104","article-title":"Nitric oxide release combined with nonsteroidal antiinflammatory activity prevents muscular dystrophy pathology and enhances stem cell therapy","volume":"104","author":"Brunelli","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2021070819095474800_ref52","doi-asserted-by":"crossref","first-page":"2670","DOI":"10.1093\/brain\/awu210","article-title":"Sialyllactose ameliorates myopathic phenotypes in symptomatic GNE myopathy model mice","volume":"137","author":"Yonekawa","year":"2014","journal-title":"Brain"},{"key":"2021070819095474800_ref53","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/0022-510X(90)90005-8","article-title":"Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions","volume":"100","author":"Weller","year":"1990","journal-title":"J. Neurol. Sci."},{"key":"2021070819095474800_ref54","doi-asserted-by":"crossref","DOI":"10.14814\/phy2.13998","article-title":"Effects of Nrf2 deficiency on mitochondrial oxidative stress in aged skeletal muscle","volume":"7","author":"Kitaoka","year":"2019","journal-title":"Physiol. Rep."},{"key":"2021070819095474800_ref55","doi-asserted-by":"crossref","first-page":"3593","DOI":"10.1016\/j.celrep.2018.08.091","article-title":"Mitochondrial diagnostics: A multiplexed assay platform for comprehensive assessment of mitochondrial energy fluxes","volume":"24","author":"Fisher-Wellman","year":"2018","journal-title":"Cell Rep."},{"key":"2021070819095474800_ref56","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1093\/bioinformatics\/btp616","article-title":"edgeR: a Bioconductor package for differential expression analysis of digital gene expression data","volume":"26","author":"Robinson","year":"2010","journal-title":"Bioinformatics"},{"key":"2021070819095474800_ref57","doi-asserted-by":"crossref","first-page":"4288","DOI":"10.1093\/nar\/gks042","article-title":"Differential expression analysis of multifactor RNA-Seq experiments with respect to biological variation","volume":"40","author":"McCarthy","year":"2012","journal-title":"Nucleic Acids Res."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/advance-article-pdf\/doi\/10.1093\/hmg\/ddab100\/37761497\/ddab100.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/30\/11\/1006\/38445682\/ddab100.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/30\/11\/1006\/38445682\/ddab100.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,9]],"date-time":"2021-07-09T02:43:46Z","timestamp":1625798626000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/30\/11\/1006\/6210386"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,5]]},"references-count":57,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2021,4,5]]},"published-print":{"date-parts":[[2021,5,31]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddab100","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,6,1]]},"published":{"date-parts":[[2021,4,5]]}}}