{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T18:02:44Z","timestamp":1794333764210,"version":"4.9.0"},"reference-count":47,"publisher":"Elsevier BV","issue":"7","license":[{"start":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T00:00:00Z","timestamp":1601510400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T00:00:00Z","timestamp":1601510400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,9,22]],"date-time":"2020-09-22T00:00:00Z","timestamp":1600732800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cell Reports Medicine"],"published-print":{"date-parts":[[2020,10]]},"DOI":"10.1016\/j.xcrm.2020.100122","type":"journal-article","created":{"date-parts":[[2020,10,20]],"date-time":"2020-10-20T19:51:50Z","timestamp":1603223510000},"page":"100122","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":11,"title":["A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKII\u03b2 Signaling in Limb Girdle Muscular Dystrophy"],"prefix":"10.1016","volume":"1","author":[{"given":"Jian","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesus","family":"Campagna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Varghese","family":"John","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Damoiseaux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekaterina","family":"Mokhonova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Becerra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huan","family":"Meng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth M.","family":"McNally","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"April D.","family":"Pyle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irina","family":"Kramerova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7104-544X","authenticated-orcid":false,"given":"Melissa J.","family":"Spencer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.xcrm.2020.100122_bib1","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1016\/j.nmd.2018.05.007","article-title":"229th ENMC international workshop: limb girdle muscular dystrophies - nomenclature and reformed classification Naarden, the Netherlands, 17-19 March 2017","volume":"28","author":"Straub","year":"2018","journal-title":"Neuromuscul. Disord."},{"key":"10.1016\/j.xcrm.2020.100122_bib2","doi-asserted-by":"crossref","first-page":"7874","DOI":"10.1002\/jcp.27907","article-title":"Prevalence, pathological mechanisms, and genetic basis of limb-girdle muscular dystrophies: a review","volume":"234","author":"Taghizadeh","year":"2019","journal-title":"J.\u00a0Cell. Physiol."},{"key":"10.1016\/j.xcrm.2020.100122_bib3","doi-asserted-by":"crossref","first-page":"2154","DOI":"10.1093\/brain\/aww133","article-title":"A heterozygous 21-bp deletion in CAPN3 causes dominantly inherited limb girdle muscular dystrophy","volume":"139","author":"Vissing","year":"2016","journal-title":"Brain"},{"key":"10.1016\/j.xcrm.2020.100122_bib4","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1002\/acn3.287","article-title":"Natural history of LGMD2A for delineating outcome measures in clinical trials","volume":"3","author":"Richard","year":"2016","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"10.1016\/j.xcrm.2020.100122_bib5","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1111\/nan.12034","article-title":"Muscle atrophy in Limb Girdle Muscular Dystrophy 2A: a morphometric and molecular study","volume":"39","author":"Fanin","year":"2013","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.xcrm.2020.100122_bib6","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1002\/mus.24682","article-title":"Protein and genetic diagnosis of limb girdle muscular dystrophy type 2A: The yield and the pitfalls","volume":"52","author":"Fanin","year":"2015","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.xcrm.2020.100122_bib7","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1002\/humu.20642","article-title":"Clinical, molecular, and protein correlations in a large sample of genetically diagnosed Italian limb girdle muscular dystrophy patients","volume":"29","author":"Guglieri","year":"2008","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.xcrm.2020.100122_bib8","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1093\/jnen\/nly113","article-title":"Divergent Features of Mitochondrial Deficiencies in LGMD2A Associated With Novel Calpain-3 Mutations","volume":"78","author":"El-Khoury","year":"2019","journal-title":"J.\u00a0Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.xcrm.2020.100122_bib9","doi-asserted-by":"crossref","first-page":"2125","DOI":"10.1093\/hmg\/ddi217","article-title":"Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway","volume":"14","author":"Kramerova","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib10","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1093\/hmg\/ddh153","article-title":"Null mutation of calpain 3 (p94) in mice causes abnormal sarcomere formation in\u00a0vivo and in\u00a0vitro","volume":"13","author":"Kramerova","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib11","doi-asserted-by":"crossref","first-page":"3194","DOI":"10.1093\/hmg\/ddp257","article-title":"Mitochondrial abnormalities, energy deficit and oxidative stress are features of calpain 3 deficiency in skeletal muscle","volume":"18","author":"Kramerova","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib12","doi-asserted-by":"crossref","first-page":"3193","DOI":"10.1093\/hmg\/dds144","article-title":"Impaired calcium calmodulin kinase signaling and muscle adaptation response in the absence of calpain 3","volume":"21","author":"Kramerova","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib13","doi-asserted-by":"crossref","first-page":"2194","DOI":"10.1093\/hmg\/ddw086","article-title":"Failure to up-regulate transcription of genes necessary for muscle adaptation underlies limb girdle muscular dystrophy 2A (calpainopathy)","volume":"25","author":"Kramerova","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib14","doi-asserted-by":"crossref","first-page":"3271","DOI":"10.1093\/hmg\/ddn223","article-title":"Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle","volume":"17","author":"Kramerova","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib15","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1146\/annurev.ph.56.030194.002453","article-title":"Structure and development of E-C coupling units in skeletal muscle","volume":"56","author":"Franzini-Armstrong","year":"1994","journal-title":"Annu. Rev. Physiol."},{"key":"10.1016\/j.xcrm.2020.100122_bib16","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1152\/japplphysiol.00015.2005","article-title":"Role of Ca2+\/calmodulin-dependent kinases in skeletal muscle plasticity","volume":"99","author":"Chin","year":"2005","journal-title":"J.\u00a0Appl. Physiol. (1985)"},{"key":"10.1016\/j.xcrm.2020.100122_bib17","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.1093\/hmg\/ddy071","article-title":"Calpain 3 and CaMKII\u03b2 signaling are required to induce HSP70 necessary for adaptive muscle growth after atrophy","volume":"27","author":"Kramerova","year":"2018","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib18","doi-asserted-by":"crossref","first-page":"e12462","DOI":"10.14814\/phy2.12462","article-title":"Exercise-induced metabolic fluctuations influence AMPK, p38-MAPK and CaMKII phosphorylation in human skeletal muscle","volume":"3","author":"Combes","year":"2015","journal-title":"Physiol. Rep."},{"key":"10.1016\/j.xcrm.2020.100122_bib19","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1113\/jphysiol.2003.054171","article-title":"Exercise increases Ca2+-calmodulin-dependent protein kinase II activity in human skeletal muscle","volume":"553","author":"Rose","year":"2003","journal-title":"J.\u00a0Physiol."},{"key":"10.1016\/j.xcrm.2020.100122_bib20","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1113\/jphysiol.2007.138529","article-title":"Effect of endurance exercise training on Ca2+ calmodulin-dependent protein kinase II expression and signalling in skeletal muscle of humans","volume":"583","author":"Rose","year":"2007","journal-title":"J.\u00a0Physiol."},{"key":"10.1016\/j.xcrm.2020.100122_bib21","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1242\/jeb.128207","article-title":"Molecular networks in skeletal muscle plasticity","volume":"219","author":"Hoppeler","year":"2016","journal-title":"J.\u00a0Exp. Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib22","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/s00424-010-0834-0","article-title":"PGC-1alpha-mediated adaptations in skeletal muscle","volume":"460","author":"Olesen","year":"2010","journal-title":"Pflugers Arch."},{"key":"10.1016\/j.xcrm.2020.100122_bib23","doi-asserted-by":"crossref","first-page":"4131","DOI":"10.1242\/dev.008367","article-title":"MEF2: a central regulator of diverse developmental programs","volume":"134","author":"Potthoff","year":"2007","journal-title":"Development"},{"key":"10.1016\/j.xcrm.2020.100122_bib24","doi-asserted-by":"crossref","first-page":"604","DOI":"10.3389\/fphar.2017.00604","article-title":"Ca2+\/Calmodulin-Dependent Protein Kinase II and Androgen Signaling Pathways Modulate MEF2 Activity in Testosterone-Induced Cardiac Myocyte Hypertrophy","volume":"8","author":"Duran","year":"2017","journal-title":"Front. Pharmacol."},{"key":"10.1016\/j.xcrm.2020.100122_bib25","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.devcel.2006.04.009","article-title":"A temporal map of transcription factor activity: mef2 directly regulates target genes at all stages of muscle development","volume":"10","author":"Sandmann","year":"2006","journal-title":"Dev. Cell"},{"key":"10.1016\/j.xcrm.2020.100122_bib26","doi-asserted-by":"crossref","first-page":"18142","DOI":"10.1016\/S0021-9258(19)84688-4","article-title":"Structure, organization, and expression of the rat cardiac myosin light chain-2 gene. Identification of a 250-base pair fragment which confers cardiac-specific expression","volume":"264","author":"Henderson","year":"1989","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.xcrm.2020.100122_bib27","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1007\/BF00354303","article-title":"An STS map of the limb girdle muscular dystrophy type 2A region","volume":"6","author":"Richard","year":"1995","journal-title":"Mamm. Genome"},{"key":"10.1016\/j.xcrm.2020.100122_bib28","doi-asserted-by":"crossref","first-page":"2512","DOI":"10.1038\/s41436-019-0544-8","article-title":"Estimating prevalence for limb-girdle muscular dystrophy based on public sequencing databases","volume":"21","author":"Liu","year":"2019","journal-title":"Genet. Med."},{"key":"10.1016\/j.xcrm.2020.100122_bib29","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.jmb.2011.01.057","article-title":"Non-proteolytic functions of calpain-3 in sarcoplasmic reticulum in skeletal muscles","volume":"407","author":"Ojima","year":"2011","journal-title":"J.\u00a0Mol. Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib30","doi-asserted-by":"crossref","first-page":"340346","DOI":"10.1155\/2009\/340346","article-title":"Alteration of sarcoplasmic reticulum ca release in skeletal muscle from calpain 3-deficient mice","volume":"2009","author":"Dayanithi","year":"2009","journal-title":"Int. J. Cell Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib31","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1186\/s13023-020-01384-x","article-title":"Preclinical model systems of ryanodine receptor 1-related myopathies and malignant hyperthermia: a comprehensive scoping review of works published 1990-2019","volume":"15","author":"Lawal","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.xcrm.2020.100122_bib32","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1210\/en.2017-00850","article-title":"\u03b2-Catenin Directs Long-Chain Fatty Acid Catabolism in the Osteoblasts of Male Mice","volume":"159","author":"Frey","year":"2018","journal-title":"Endocrinology"},{"key":"10.1016\/j.xcrm.2020.100122_bib33","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1093\/biolre\/iox048","article-title":"Consequences of endogenous and exogenous WNT signaling for development of the preimplantation bovine embryo","volume":"96","author":"Tribulo","year":"2017","journal-title":"Biol. Reprod."},{"key":"10.1016\/j.xcrm.2020.100122_bib34","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1177\/1479164117738442","article-title":"Interrelationship of canonical and non-canonical Wnt signalling pathways in chronic metabolic diseases","volume":"15","author":"Ackers","year":"2018","journal-title":"Diab. Vasc. Dis. Res."},{"key":"10.1016\/j.xcrm.2020.100122_bib35","doi-asserted-by":"crossref","first-page":"e2","DOI":"10.1017\/erm.2017.3","article-title":"FRZB and melusin, overexpressed in LGMD2A, regulate integrin \u03b21D isoform replacement altering myoblast fusion and the integrin-signalling pathway","volume":"19","author":"Jaka","year":"2017","journal-title":"Expert Rev. Mol. Med."},{"key":"10.1016\/j.xcrm.2020.100122_bib36","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.ceca.2005.06.022","article-title":"Wnt and calcium signaling: beta-catenin-independent pathways","volume":"38","author":"Kohn","year":"2005","journal-title":"Cell Calcium"},{"key":"10.1016\/j.xcrm.2020.100122_bib37","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.celrep.2017.09.023","article-title":"A Wnt\/Calcium Signaling Cascade Regulates Neuronal Excitability and Trafficking of NMDARs","volume":"21","author":"McQuate","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.xcrm.2020.100122_bib38","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.tcb.2012.07.008","article-title":"Wnt signaling in myogenesis","volume":"22","author":"von Maltzahn","year":"2012","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib39","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1038\/ncb2404","article-title":"Wnt7a-Fzd7 signalling directly activates the Akt\/mTOR anabolic growth pathway in skeletal muscle","volume":"14","author":"von Maltzahn","year":"2011","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib40","doi-asserted-by":"crossref","first-page":"20614","DOI":"10.1073\/pnas.1215765109","article-title":"Wnt7a treatment ameliorates muscular dystrophy","volume":"109","author":"von Maltzahn","year":"2012","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.xcrm.2020.100122_bib41","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1002\/cm.21496","article-title":"The small molecule AMBMP disrupts microtubule growth, ciliogenesis, cell polarity, and cell migration","volume":"75","author":"Werner","year":"2018","journal-title":"Cytoskeleton (Hoboken)"},{"key":"10.1016\/j.xcrm.2020.100122_bib42","doi-asserted-by":"crossref","first-page":"4407","DOI":"10.2527\/jas2017.1927","article-title":"Consequences of exposure of embryos produced in\u00a0vitro in a serum-containing medium to dickkopf-related protein 1 and colony stimulating factor 2 on blastocyst yield, pregnancy rate, and birth weight","volume":"95","author":"Tr\u00edbulo","year":"2017","journal-title":"J.\u00a0Anim. Sci."},{"key":"10.1016\/j.xcrm.2020.100122_bib43","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1530\/REP-16-0610","article-title":"WNT regulation of embryonic development likely involves pathways independent of nuclear CTNNB1","volume":"153","author":"Tribulo","year":"2017","journal-title":"Reproduction"},{"key":"10.1016\/j.xcrm.2020.100122_bib44","doi-asserted-by":"crossref","first-page":"3331","DOI":"10.1093\/hmg\/ddr239","article-title":"Pathogenity of some limb girdle muscular dystrophy mutations can result from reduced anchorage to myofibrils and altered stability of calpain 3","volume":"20","author":"Ermolova","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.xcrm.2020.100122_bib45","doi-asserted-by":"crossref","first-page":"214","DOI":"10.5114\/ninp.2013.35490","article-title":"Muscle pathology in 31 patients with calpain 3 gene mutations","volume":"47","author":"Nadaj-Pakleza","year":"2013","journal-title":"Neurol. Neurochir. Pol."},{"key":"10.1016\/j.xcrm.2020.100122_bib46","doi-asserted-by":"crossref","first-page":"8437","DOI":"10.1128\/MCB.01296-06","article-title":"Regulation of the M-cadherin-beta-catenin complex by calpain 3 during terminal stages of myogenic differentiation","volume":"26","author":"Kramerova","year":"2006","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.xcrm.2020.100122_bib47","doi-asserted-by":"crossref","first-page":"e104073","DOI":"10.15252\/embj.2019104073","article-title":"A novel approach to measure mitochondrial respiration in frozen biological samples","volume":"39","author":"Acin-Perez","year":"2020","journal-title":"EMBO J."}],"container-title":["Cell Reports Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2666379120301646?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2666379120301646?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T22:46:07Z","timestamp":1762987567000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2666379120301646"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10]]},"references-count":47,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["S2666379120301646"],"URL":"https:\/\/doi.org\/10.1016\/j.xcrm.2020.100122","relation":{},"ISSN":["2666-3791"],"issn-type":[{"value":"2666-3791","type":"print"}],"subject":[],"published":{"date-parts":[[2020,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKII\u03b2 Signaling in Limb Girdle Muscular Dystrophy","name":"articletitle","label":"Article Title"},{"value":"Cell Reports Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.xcrm.2020.100122","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Authors.","name":"copyright","label":"Copyright"}],"article-number":"100122"}}