{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T11:06:22Z","timestamp":1789124782064,"version":"build-2803163510"},"reference-count":42,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T00:00:00Z","timestamp":1789084800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T00:00:00Z","timestamp":1789084800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Autosomal dominant centronuclear myopathy (ADCNM), most commonly caused by mutations in the\n                      <jats:italic>dynamin 2<\/jats:italic>\n                      (\n                      <jats:italic>DNM2<\/jats:italic>\n                      ) gene, is a rare congenital myopathy characterized by progressive muscle weakness and atrophy. Myostatin, a key negative regulator of skeletal muscle mass, has shown therapeutic potential in several models of neuromuscular diseases. We hypothesized that inhibiting myostatin could counteract muscle deconditioning in ADCNM and evaluated the therapeutic potential of both genetic and pharmacological myostatin inhibition strategies in a mouse model of ADCNM.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Knockin\u2010\n                      <jats:italic>dnm2<\/jats:italic>\n                      <jats:sup>R465W\/+<\/jats:sup>\n                      (KI) mice were first crossed with knockout\u2010\n                      <jats:italic>myostatin<\/jats:italic>\n                      mice (KO) to generate double mutant (KIKO) mice carrying both the R465W missense mutation and the\n                      <jats:italic>myostatin<\/jats:italic>\n                      gene deletion. In a second experiment, KI mice received intraperitoneal injections of a soluble activin type IIB receptor fused to IgG1 Fc fragment (sActRIIB\u2010Fc) at 5\u2009mg\u2009kg\n                      <jats:sup>\u22121<\/jats:sup>\n                      twice weekly starting at 4\u2009weeks of age for 4\u2009weeks. Muscle structure, molecular pathways and functional analysis were performed in\n                      <jats:italic>tibialis anterior<\/jats:italic>\n                      muscle.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      MRI and immunohistochemical analyses showed that KI mice exhibited impaired postnatal muscle growth between 1 and 2\u2009months of age, resulting in persistent muscle hypotrophy (\u221215%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05). This defect was associated with a significant reduction in the number of satellite cells (\u221253%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001 at 1\u2009month and \u221268%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01 at 2\u2009months), the central accumulation of dense NADH\u2010TR staining in more than 20% of muscle fibres, an upregulation of atrophy\u2010related E3 ligases mRNA (\n                      <jats:italic>Trim63<\/jats:italic>\n                      \u2009+\u200950%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001\n                      <jats:italic>and<\/jats:italic>\n                      +87%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001\n                      <jats:italic>; Fbxo32\u2009+\u200939% p<\/jats:italic>\n                      \u2009&lt;\u20090.05\n                      <jats:italic>and<\/jats:italic>\n                      +95%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001 at 1 and 2\u2009months of age, respectively) and impaired autophagy. Genetic deletion of\n                      <jats:italic>myostatin<\/jats:italic>\n                      in KIKO mice fully restored muscle mass and normalized muscle function to wild\u2010type conditions. Pharmacological inhibition with sActRIIB\u2010Fc also robustly restored muscle mass to wild\u2010type values, primarily via activation of the Akt\u2013mTOR pathway. However, this anabolic effect was transient, as muscle mass returned to baseline 5\u2009weeks after treatment cessation. Importantly, despite increased muscle mass, sActRIIB\u2010Fc treatment did not improve muscle force and key pathological features, including defects in proteostasis, mitochondrial organization and excitation\u2010contraction coupling.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These findings establish the proof of concept that myostatin inhibition counteracts skeletal muscle growth defect in ADCNM, which may be combined with other drugs to better address the multifactorial nature of muscle weakness in ADCNM.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jcsm.70338","type":"journal-article","created":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T10:13:23Z","timestamp":1789121603000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Genetic and Pharmacologic Inhibition of Myostatin Restores Muscle Mass in a\n                    <scp>\n                      <i>Dynamin 2<\/i>\n                    <\/scp>\n                    \u2010Related Centronuclear Myopathy Mouse Model"],"prefix":"10.1002","volume":"17","author":[{"given":"Durieux","family":"Anne\u2010C\u00e9cile","sequence":"first","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnould","family":"David","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velarde","family":"Mathias","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allibert","family":"Valentine","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paret","family":"Chlo\u00e9","sequence":"additional","affiliation":[{"name":"Laboratoire Physiopathologie et G\u00e9n\u00e9tique du Neurone et du Muscle (PGNM), Institut NeuroMyoGene (INMG) Univ Lyon, Universit\u00e9 Lyon 1  France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debelley","family":"Quentin","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kizilkilic","family":"Esra","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chambon","family":"Sidney","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Girard","family":"Emmanuelle","sequence":"additional","affiliation":[{"name":"Laboratoire Physiopathologie et G\u00e9n\u00e9tique du Neurone et du Muscle (PGNM), Institut NeuroMyoGene (INMG) Univ Lyon, Universit\u00e9 Lyon 1  France"},{"name":"Aniphy, SFR Sant\u00e9 Lyon\u2010Est Univ Lyon, Universit\u00e9 Lyon1  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Collin","family":"Louise","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gensel","family":"Alexandre","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Castells","family":"Josiane","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasternack","family":"Aja","sequence":"additional","affiliation":[{"name":"Department of Physiology, Faculty of Medicine University of Helsinki  Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bowen","family":"Maximilien","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morel","family":"Baptiste","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hourd\u00e9","family":"Christophe","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Schaeffer","sequence":"additional","affiliation":[{"name":"Laboratoire Physiopathologie et G\u00e9n\u00e9tique du Neurone et du Muscle (PGNM), Institut NeuroMyoGene (INMG) Univ Lyon, Universit\u00e9 Lyon 1  France"},{"name":"Centre de Biotechnologie Cellulaire Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ritvos","family":"Olli","sequence":"additional","affiliation":[{"name":"Department of Physiology, Faculty of Medicine University of Helsinki  Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bitoun","family":"Marc","sequence":"additional","affiliation":[{"name":"Inserm, Centre de Recherche en Myologie, Institut de Myologie Sorbonne Universit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mounier","family":"R\u00e9mi","sequence":"additional","affiliation":[{"name":"Laboratoire Physiopathologie et G\u00e9n\u00e9tique du Neurone et du Muscle (PGNM), Institut NeuroMyoGene (INMG) Univ Lyon, Universit\u00e9 Lyon 1  France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6130-8207","authenticated-orcid":false,"given":"Freyssenet","family":"Damien","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 Universit\u00e9 Jean Monnet Saint\u2010Etienne, Universit\u00e9 Lyon 1, Universit\u00e9 Savoie Mont\u2010Blanc  Saint\u2010Etienne France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,11]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"crossref","DOI":"10.3390\/ijms222111377","article-title":"Common Pathogenic Mechanisms in Centronuclear and Myotubular Myopathies and Latest Treatment Advances","volume":"22","author":"Gomez\u2010Oca R.","year":"2021","journal-title":"International Journal of Molecular Sciences"},{"key":"e_1_2_9_3_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1001\/archneur.1966.00470070005001","article-title":"Myotubular Myopathy: Persistence of Fetal Muscle in an Adolescent Boy","volume":"14","author":"Spiro A. J.","year":"1966","journal-title":"Archives of Neurology"},{"key":"e_1_2_9_4_1","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1038\/ng1657","article-title":"Mutations in Dynamin 2 Cause Dominant Centronuclear Myopathy","volume":"37","author":"Bitoun M.","year":"2005","journal-title":"Nature Genetics"},{"issue":"154","key":"e_1_2_9_5_1","article-title":"A DNM2 Centronuclear Myopathy Mutation Reveals a Link Between Recycling Endosome Scission and Autophagy","volume":"53","author":"Puri C.","year":"2020","journal-title":"Developmental Cell"},{"key":"e_1_2_9_6_1","doi-asserted-by":"crossref","DOI":"10.3389\/fgene.2025.1559773","article-title":"A Novel DNM2 Variant Associated With Centronuclear Myopathy: A Case Report","volume":"16","author":"Rimoldi M.","year":"2025","journal-title":"Frontiers in Genetics"},{"key":"e_1_2_9_7_1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0027498","article-title":"Mild Functional Differences of Dynamin 2 Mutations Associated to Centronuclear Myopathy and Charcot\u2010Marie Tooth Peripheral Neuropathy","volume":"6","author":"Koutsopoulos O. S.","year":"2011","journal-title":"PLoS ONE"},{"key":"e_1_2_9_8_1","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1016\/j.nmd.2018.06.012","article-title":"An Integrated Modelling Methodology for Estimating the Prevalence of Centronuclear Myopathy","volume":"28","author":"Vandersmissen I.","year":"2018","journal-title":"Neuromuscular Disorders"},{"key":"e_1_2_9_9_1","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1038\/387083a0","article-title":"Regulation of Skeletal Muscle Mass in Mice by a New TGF\u2010p Superfamily Member","volume":"387","author":"McPherron A. C.","year":"1997","journal-title":"Nature"},{"key":"e_1_2_9_10_1","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1210\/en.2008-0959","article-title":"Down\u2010Regulation of Akt\/Mammalian Target of Rapamycin Signaling Pathway in Response to Myostatin Overexpression in Skeletal Muscle","volume":"150","author":"Amirouche A.","year":"2009","journal-title":"Endocrinology"},{"key":"e_1_2_9_11_1","doi-asserted-by":"crossref","first-page":"3140","DOI":"10.1210\/en.2006-1500","article-title":"Ectopic Expression of Myostatin Induces Atrophy of Adult Skeletal Muscle by Decreasing Muscle Gene Expression","volume":"148","author":"Durieux A. C.","year":"2007","journal-title":"Endocrinology"},{"key":"e_1_2_9_12_1","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1146\/annurev-physiol-012422-112116","article-title":"Myostatin: A Skeletal Muscle Chalone","volume":"85","author":"Lee S.\u2010J.","year":"2023","journal-title":"Annual Review of Physiology"},{"key":"e_1_2_9_13_1","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1083\/jcb.200207056","article-title":"Myostatin Negatively Regulates Satellite Cell Activation and Self\u2010Renewal","volume":"162","author":"McCroskery S.","year":"2003","journal-title":"Journal of Cell Biology"},{"key":"e_1_2_9_14_1","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1002\/jcp.20757","article-title":"Myostatin Induces cachexia by Activating the Ubiquitin Proteolytic System Through an NF\u2010\u03baB\u2010Independent, @1\u2010Dependent Mechanism","volume":"209","author":"McFarlane C.","year":"2006","journal-title":"Journal of Cellular Physiology"},{"key":"e_1_2_9_15_1","doi-asserted-by":"crossref","first-page":"533","DOI":"10.3390\/cells10030533","article-title":"Antimyostatin Treatment in Health and Disease: The Story of Great Expectations and Limited Success","volume":"10","author":"Nielsen T. L.","year":"2021","journal-title":"Cells"},{"key":"e_1_2_9_16_1","doi-asserted-by":"crossref","first-page":"1831","DOI":"10.1016\/j.ajpath.2014.03.003","article-title":"Differential Muscle Hypertrophy Is Associated With Satellite Cell Numbers and Akt Pathway Activation Following Activin Type IIB Receptor Inhibition in Mtm1 p.R69C Mice","volume":"184","author":"Lawlor M. W.","year":"2014","journal-title":"American Journal of Pathology"},{"key":"e_1_2_9_17_1","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1016\/j.ajpath.2010.10.035","article-title":"Inhibition of Activin Receptor Type IIB Increases Strength and Lifespan in Myotubularin\u2010Deficient Mice","volume":"178","author":"Lawlor M. W.","year":"2011","journal-title":"American Journal of Pathology"},{"key":"e_1_2_9_18_1","doi-asserted-by":"crossref","first-page":"4820","DOI":"10.1093\/hmg\/ddq413","article-title":"A Centronuclear Myopathy\u2010Dynamin 2 Mutation Impairs Skeletal Muscle Structure and Function in Mice","volume":"19","author":"Durieux A. C.","year":"2010","journal-title":"Human Molecular Genetics"},{"key":"e_1_2_9_19_1","doi-asserted-by":"crossref","first-page":"7344","DOI":"10.1158\/0008-5472.CAN-14-0057","article-title":"Myostatin Gene Inactivation Prevents Skeletal Muscle Wasting in Cancer","volume":"74","author":"Gallot Y. S.","year":"2014","journal-title":"Cancer Research"},{"key":"e_1_2_9_20_1","doi-asserted-by":"crossref","first-page":"14000","DOI":"10.1038\/s41598-017-13912-0","article-title":"Pharmacological Inhibition of Myostatin Improves Skeletal Muscle Mass and Function in a Mouse Model of Stroke","volume":"7","author":"Desgeorges M. M.","year":"2017","journal-title":"Scientific Reports"},{"key":"e_1_2_9_21_1","doi-asserted-by":"crossref","DOI":"10.1111\/apha.13034","article-title":"Specific Labelling of Myonuclei by an Antibody Against Pericentriolar Material 1 on Skeletal Muscle Tissue Sections","volume":"223","author":"Winje I. M.","year":"2018","journal-title":"Acta Physiologica"},{"key":"e_1_2_9_22_1","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1152\/physrev.1972.52.1.129","article-title":"Dynamic Properties of Mammalian Skeletal Muscles","volume":"52","author":"Close R. I.","year":"1972","journal-title":"Physiological Reviews"},{"key":"e_1_2_9_23_1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0147669","article-title":"Musculoskeletal Geometry, Muscle Architecture and Functional Specialisations of the Mouse Hindlimb","volume":"11","author":"Charles J. P.","year":"2016","journal-title":"PLoS ONE"},{"key":"e_1_2_9_24_1","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.nmd.2012.12.009","article-title":"Centronuclear myopathy Related to Dynamin 2 Mutations: Clinical, Morphological, Muscle Imaging and Genetic Features of an Italian Cohort","volume":"23","author":"Catteruccia M.","year":"2013","journal-title":"Neuromuscular Disorders"},{"key":"e_1_2_9_25_1","doi-asserted-by":"crossref","first-page":"2224","DOI":"10.1016\/j.ajpath.2011.01.054","article-title":"Increased Expression of Wild\u2010Type or a Centronuclear Myopathy Mutant of Dynamin 2 in Skeletal Muscle of Adult Mice Leads to Structural Defects and Muscle Weakness","volume":"178","author":"Cowling B. S.","year":"2011","journal-title":"American Journal of Pathology"},{"key":"e_1_2_9_26_1","doi-asserted-by":"crossref","first-page":"49831","DOI":"10.1074\/jbc.M204291200","article-title":"Myostatin Inhibits Myoblast Differentiation by Down\u2010Regulating MyoD Expression","volume":"277","author":"Langley B.","year":"2002","journal-title":"Journal of Biological Chemistry"},{"key":"e_1_2_9_27_1","doi-asserted-by":"crossref","first-page":"40235","DOI":"10.1074\/jbc.M004356200","article-title":"Myostatin, a Negative Regulator of Muscle Growth, Functions by Inhibiting Myoblast Proliferation","volume":"275","author":"Thomas M.","year":"2000","journal-title":"Journal of Biological Chemistry"},{"key":"e_1_2_9_28_1","doi-asserted-by":"crossref","first-page":"E41","DOI":"10.1152\/ajpendo.00389.2012","article-title":"Muscle Protein Synthesis, mTORC1\/MAPK\/Hippo Signaling, and Capillary Density Are Altered by Blocking of Myostatin and Activins","volume":"304","author":"Hulmi J. J.","year":"2013","journal-title":"American Journal of Physiology. Endocrinology and Metabolism"},{"key":"e_1_2_9_29_1","doi-asserted-by":"crossref","first-page":"4417","DOI":"10.1093\/hmg\/ddt292","article-title":"Muscle\u2010Specific Function of the Centronuclear Myopathy and Charcot\u2010Marie\u2010Tooth Neuropathy\u2010Associated Dynamin 2 Is Required for Proper Lipid Metabolism, Mitochondria, Muscle Fibers, Neuromuscular Junctions, and Peripheral Nerves","volume":"22","author":"Tinelli E.","year":"2013","journal-title":"Human Molecular Genetics"},{"key":"e_1_2_9_30_1","doi-asserted-by":"crossref","first-page":"dev167197","DOI":"10.1242\/dev.167197","article-title":"Prepubertal Skeletal Muscle Growth Requires Pax7\u2010Expressing Satellite Cell\u2010Derived Myonuclear Contribution","volume":"145","author":"Bachman J. F.","year":"2018","journal-title":"Development"},{"key":"e_1_2_9_31_1","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1038\/s41598-018-38184-0","article-title":"Nuclear Defects in Skeletal Muscle From a Dynamin 2\u2010Linked Centronuclear Myopathy Mouse Model","volume":"9","author":"Fongy A.","year":"2019","journal-title":"Scientific Reports"},{"key":"e_1_2_9_32_1","doi-asserted-by":"crossref","first-page":"18763","DOI":"10.1073\/pnas.0900705106","article-title":"T\u2010Tubule Disorganization and Defective Excitation\u2010Contraction Coupling in Muscle Fibers Lacking Myotubularin Lipid Phosphatase","volume":"106","author":"Al\u2010Qusairi L.","year":"2009","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"e_1_2_9_33_1","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1038\/s41467-017-01486-4","article-title":"Downregulation of Myostatin Pathway in Neuromuscular Diseases May Explain Challenges of Anti\u2010Myostatin Therapeutic Approaches","volume":"8","author":"Mariot V.","year":"2017","journal-title":"Nature Communications"},{"key":"e_1_2_9_34_1","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/s13395-018-0180-z","article-title":"Myostatin and Activin Blockade by Engineered Follistatin Results in Hypertrophy and Improves Dystrophic Pathology in mdx Mouse More Than Myostatin Blockade Alone","volume":"8","author":"Iskenderian A.","year":"2018","journal-title":"Skeletal Muscle"},{"key":"e_1_2_9_35_1","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1002\/mus.21743","article-title":"Activin IIB Receptor Blockade Attenuates Dystrophic Pathology in a Mouse Model of Duchenne Muscular Dystrophy","volume":"42","author":"Morine K. J.","year":"2010","journal-title":"Muscle and Nerve"},{"key":"e_1_2_9_36_1","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/j.ajpath.2010.11.071","article-title":"Targeting the Activin Type IIB Receptor to Improve Muscle Mass and Function in the mdx Mouse Model of Duchenne Muscular Dystrophy","volume":"178","author":"Pistilli E. E.","year":"2011","journal-title":"American Journal of Pathology"},{"key":"e_1_2_9_37_1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0166803","article-title":"Activin Receptor Type IIB Inhibition Improves Muscle Phenotype and Function in a Mouse Model of Spinal Muscular Atrophy","volume":"11","author":"Liu M.","year":"2016","journal-title":"PLoS ONE"},{"key":"e_1_2_9_38_1","doi-asserted-by":"crossref","first-page":"1568","DOI":"10.1016\/j.ajpath.2016.02.008","article-title":"Treatment With ActRIIB\u2010mFc Produces Myofiber Growth and Improves Lifespan in the Acta1 H40Y Murine Model of Nemaline Myopathy","volume":"186","author":"Tinklenberg J.","year":"2016","journal-title":"American Journal of Pathology"},{"key":"e_1_2_9_39_1","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1242\/bio.020263","article-title":"Calcium Homeostasis Alterations in a Mouse Model of the Dynamin 2\u2010Related Centronuclear Myopathy","volume":"5","author":"Fraysse B.","year":"2016","journal-title":"Biol Open"},{"key":"e_1_2_9_40_1","doi-asserted-by":"crossref","first-page":"7369","DOI":"10.1113\/JP274990","article-title":"Impaired Excitation\u2010Contraction Coupling in Muscle Fibres From the Dynamin2(R465W) Mouse Model of Centronuclear Myopathy","volume":"595","author":"Kutchukian C.","year":"2017","journal-title":"Journal of Physiology"},{"key":"e_1_2_9_41_1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0025740","article-title":"Disrupted Membrane Structure and Intracellular Ca2+ Signaling in Adult Skeletal Muscle With Acute Knockdown of Bin1","volume":"6","author":"Tjondrokoesoemo A.","year":"2011","journal-title":"PLoS ONE"},{"key":"e_1_2_9_42_1","doi-asserted-by":"crossref","first-page":"5542","DOI":"10.1093\/hmg\/ddv285","article-title":"Dynamin\u20102 Mutations Associated With Centronuclear Myopathy Are Hypermorphic and Lead to T\u2010Tubule Fragmentation","volume":"24","author":"Chin Y. H.","year":"2015","journal-title":"Human Molecular Genetics"},{"key":"e_1_2_9_43_1","doi-asserted-by":"crossref","first-page":"15153","DOI":"10.1038\/ncomms15153","article-title":"Activin A More Prominently Regulates Muscle Mass in primates Than Does GDF8","volume":"8","author":"Latres E.","year":"2017","journal-title":"Nature Communications"}],"container-title":["Journal of Cachexia, Sarcopenia and Muscle"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcsm.70338","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jcsm.70338","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcsm.70338","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T10:13:28Z","timestamp":1789121608000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jcsm.70338"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,11]]},"references-count":42,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,10]]}},"alternative-id":["10.1002\/jcsm.70338"],"URL":"https:\/\/doi.org\/10.1002\/jcsm.70338","archive":["Portico"],"relation":{},"ISSN":["2190-5991","2190-6009"],"issn-type":[{"value":"2190-5991","type":"print"},{"value":"2190-6009","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,11]]},"assertion":[{"value":"2025-11-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-18","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70338"}}