{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T09:19:25Z","timestamp":1790759965500,"version":"4.1.0"},"reference-count":105,"publisher":"Rockefeller University Press","issue":"9","license":[{"start":{"date-parts":[[2022,8,8]],"date-time":"2022-08-08T00:00:00Z","timestamp":1659916800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AR059646"],"award-info":[{"award-number":["AR059646"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS097868"],"award-info":[{"award-number":["NS097868"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,9,5]]},"abstract":"<jats:p>Duchenne muscular dystrophy (DMD), an X-linked disorder caused by loss-of-function mutations in the dystrophin gene, is characterized by progressive muscle degeneration and weakness. Enhanced store-operated Ca2+ entry (SOCE), a Ca2+ influx mechanism coordinated by STIM1 sensors of luminal Ca2+ within the sarcoplasmic reticulum (SR) and Ca2+-permeable Orai1 channels in the sarcolemma, is proposed to contribute to Ca2+-mediated muscle damage in DMD. To directly determine the impact of Orai1-dependent SOCE on the dystrophic phenotype, we crossed mdx mice with tamoxifen-inducible, muscle-specific Orai1 knockout mice (mdx-Orai1 KO mice). Both constitutive and SOCE were significantly increased in flexor digitorum brevis fibers from mdx mice, while SOCE was absent in fibers from both Orai1 KO and mdx-Orai1 KO mice. Compared with WT mice, fibers from mdx mice exhibited (1) increased resting myoplasmic Ca2+ levels, (2) reduced total releasable Ca2+ store content, and (3) a prolonged rate of electrically evoked Ca2+ transient decay. These effects were partially normalized in fibers from mdx-Orai1 KO mice. Intact extensor digitorum longus muscles from mdx mice exhibited a significant reduction of maximal specific force, which was rescued in muscles from mdx-Orai1 KO mice. Finally, during exposure to consecutive eccentric contractions, muscles from mdx mice displayed a more pronounced decline in specific force compared with that of WT mice, which was also significantly attenuated by Orai1 ablation. Together, these results indicate that enhanced Orai1-dependent SOCE exacerbates the dystrophic phenotype and that Orai1 deficiency improves muscle pathology by both normalizing Ca2+ homeostasis and promoting sarcolemmal integrity\/stability.<\/jats:p>","DOI":"10.1085\/jgp.202213081","type":"journal-article","created":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T02:20:35Z","timestamp":1657246835000},"update-policy":"https:\/\/doi.org\/10.1085\/jgp.crossmarkpolicy","source":"Crossref","is-referenced-by-count":20,"title":["Postdevelopmental knockout of Orai1 improves muscle pathology in a mouse model of Duchenne muscular dystrophy"],"prefix":"10.1085","volume":"154","author":[{"given":"Maricela","family":"Garc\u00eda-Casta\u00f1eda","sequence":"first","affiliation":[{"name":"Department of Pharmacology and Physiology, University of Rochester School of Medicine and Dentistry, Rochester, NY 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0720-3009","authenticated-orcid":true,"given":"Antonio","family":"Michelucci","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology, University of Rochester School of Medicine and Dentistry, Rochester, NY 1"},{"name":"2Department of Chemistry, Biology and Biotechnology, University of Perugia, Perugia, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nan","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology, University of Rochester School of Medicine and Dentistry, Rochester, NY 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sundeep","family":"Malik","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology, University of Rochester School of Medicine and Dentistry, Rochester, NY 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3182-1755","authenticated-orcid":true,"given":"Robert T.","family":"Dirksen","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology, University of Rochester School of Medicine and Dentistry, Rochester, NY 1"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2022,8,8]]},"reference":[{"key":"2023072122511031800_bib1","doi-asserted-by":"publisher","first-page":"20876","DOI":"10.1074\/jbc.M112.344929","article-title":"Increased resting intracellular calcium modulates NF-\u03baB-dependent inducible nitric-oxide synthase gene expression in dystrophic mdx skeletal myotubes","volume":"287","author":"Altamirano","year":"2012","journal-title":"J. Biol. Chem."},{"key":"2023072122511031800_bib2","doi-asserted-by":"publisher","first-page":"762","DOI":"10.1073\/pnas.1013067108","article-title":"Biglycan recruits utrophin to the sarcolemma and counters dystrophic pathology in mdx mice","volume":"108","author":"Amenta","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072122511031800_bib3","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1186\/2044-5040-2-9","article-title":"Leaky ryanodine receptors in \u03b2-sarcoglycan deficient mice: A potential common defect in muscular dystrophy","volume":"2","author":"Andersson","year":"2012","journal-title":"Skelet. Muscle"},{"key":"2023072122511031800_bib4","doi-asserted-by":"publisher","first-page":"dev167197","DOI":"10.1242\/dev.167197","article-title":"Prepubertal skeletal muscle growth requires Pax7-expressing satellite cell-derived myonuclear contribution","volume":"145","author":"Bachman","year":"2018","journal-title":"Development"},{"key":"2023072122511031800_bib5","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1111\/iep.12142","article-title":"Changes in calsequestrin, TNF-\u03b1, TGF-\u03b2 and MyoD levels during the progression of skeletal muscle dystrophy in mdx mice: A comparative analysis of the quadriceps, diaphragm and intrinsic laryngeal muscles","volume":"96","author":"Barros Maranh\u00e3o","year":"2015","journal-title":"Int. J. Exp. Pathol."},{"key":"2023072122511031800_bib6","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1113\/jphysiol.2003.041608","article-title":"Sarcoplasmic reticulum calcium release compared in slow-twitch and fast-twitch fibres of mouse muscle","volume":"551","author":"Baylor","year":"2003","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib7","doi-asserted-by":"publisher","first-page":"325","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":"2023072122511031800_bib8","doi-asserted-by":"publisher","first-page":"3733","DOI":"10.1242\/JCS.03184","article-title":"Ca2+-independent phospholipase A2 enhances store-operated Ca2+ entry in dystrophic skeletal muscle fibers","volume":"119","author":"Boittin","year":"2006","journal-title":"J. Cell Sci."},{"key":"2023072122511031800_bib9","doi-asserted-by":"publisher","first-page":"14286","DOI":"10.1038\/s41598-017-14134-0","article-title":"Exercise-dependent formation of new junctions that promote STIM1-Orai1 assembly in skeletal muscle","volume":"7","author":"Boncompagni","year":"2017","journal-title":"Sci. Rep."},{"key":"2023072122511031800_bib10","doi-asserted-by":"publisher","first-page":"17463","DOI":"10.1038\/s41598-018-33063-0","article-title":"Erratum: Addendum: Exercise-dependent formation of new junctions that promote STIM1-Orai1 assembly in skeletal muscle","volume":"8","author":"Boncompagni","year":"2018","journal-title":"Sci. Rep."},{"key":"2023072122511031800_bib11","doi-asserted-by":"publisher","first-page":"3024","DOI":"10.1093\/hmg\/ddz105","article-title":"Mouse model of severe recessive RYR1-related myopathy","volume":"28","author":"Brennan","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"2023072122511031800_bib12","doi-asserted-by":"publisher","DOI":"10.26402\/jpp.2021.2.01","article-title":"The role of mitochondria in Duchenne muscular dystrophy","volume":"72","author":"Budzinska","year":"2021","journal-title":"J. Physiol. Pharmacol."},{"key":"2023072122511031800_bib105","doi-asserted-by":"publisher","first-page":"611","DOI":"10.1373\/clinchem.2008.112797","article-title":"The MIQE guidelines: Minimum information for publication of quantitative real-time PCR experiments","volume":"55","author":"Bustin","year":"2009","journal-title":"Clin. Chem"},{"key":"2023072122511031800_bib13","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1113\/jphysiol.2004.081034","article-title":"Calcium transients in developing mouse skeletal muscle fibres","volume":"564","author":"Capote","year":"2005","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib14","doi-asserted-by":"publisher","first-page":"C1077","DOI":"10.1152\/ajpcell.00428.2009","article-title":"Excitation-contraction coupling alterations in mdx and utrophin\/dystrophin double knockout mice: A comparative study","volume":"298","author":"Capote","year":"2010","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2023072122511031800_bib15","doi-asserted-by":"publisher","first-page":"4109","DOI":"10.1096\/fj.201600621R","article-title":"Orai1 enhances muscle endurance by promoting fatigue-resistant type I fiber content but not through acute store-operated Ca2+ entry","volume":"30","author":"Carrell","year":"2016","journal-title":"FASEB J."},{"key":"2023072122511031800_bib16","doi-asserted-by":"publisher","first-page":"C1122","DOI":"10.1152\/ajpcell.1999.277.6.C1122","article-title":"Calcium transients in single fibers of low-frequency stimulated fast-twitch muscle of rat","volume":"277","author":"Carroll","year":"1999","journal-title":"Am. J. Physiol."},{"key":"2023072122511031800_bib17","doi-asserted-by":"publisher","first-page":"C382","DOI":"10.1152\/ajpcell.00198.2021","article-title":"Role of SERCA and sarcolipin in adaptive muscle remodeling","volume":"322","author":"Chambers","year":"2022","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2023072122511031800_bib18","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1152\/japplphysiol.00903.2001","article-title":"Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle","volume":"92","author":"Culligan","year":"2002","journal-title":"J. Appl. Physiol."},{"key":"2023072122511031800_bib19","doi-asserted-by":"publisher","first-page":"C567","DOI":"10.1152\/ajpcell.00144.2012","article-title":"Changes in plasma membrane Ca-ATPase and stromal interacting molecule 1 expression levels for Ca2+ signaling in dystrophic mdx mouse muscle","volume":"303","author":"Cully","year":"2012","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2023072122511031800_bib20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.pediatrneurol.2006.09.016","article-title":"Pathophysiology of duchenne muscular dystrophy: Current hypotheses","volume":"36","author":"Deconinck","year":"2007","journal-title":"Pediatr. Neurol."},{"key":"2023072122511031800_bib21","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1023\/a:1014587918367","article-title":"Tibialis anterior muscles in mdx mice are highly susceptible to contraction-induced injury","volume":"22","author":"Dellorusso","year":"2001","journal-title":"J. Muscle Res. Cell Motil."},{"key":"2023072122511031800_bib22","doi-asserted-by":"publisher","first-page":"14698","DOI":"10.1073\/pnas.0802217105","article-title":"Dystrophic skeletal muscle fibers display alterations at the level of calcium microdomains","volume":"105","author":"DiFranco","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072122511031800_bib23","doi-asserted-by":"publisher","first-page":"3139","DOI":"10.1113\/jphysiol.2009.172148","article-title":"Checking your SOCCs and feet: The molecular mechanisms of Ca2+ entry in skeletal muscle","volume":"587","author":"Dirksen","year":"2009","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib24","doi-asserted-by":"publisher","first-page":"634","DOI":"10.1007\/s00424-002-0854-5","article-title":"Sarcoplasmic reticulum function in slow- and fast-twitch skeletal muscles from mdx mice","volume":"444","author":"Divet","year":"2002","journal-title":"Pflugers Arch."},{"key":"2023072122511031800_bib25","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1111\/j.1365-201X.2005.01450.x","article-title":"Effect of cyclopiazonic acid, an inhibitor of the sarcoplasmic reticulum Ca-ATPase, on skeletal muscles from normal and mdx mice","volume":"184","author":"Divet","year":"2005","journal-title":"Acta Physiol. Scand."},{"key":"2023072122511031800_bib26","doi-asserted-by":"publisher","first-page":"3943","DOI":"10.1111\/j.1432-1033.2004.04332.x","article-title":"Subproteomics analysis of Ca+-binding proteins demonstrates decreased calsequestrin expression in dystrophic mouse skeletal muscle","volume":"271","author":"Doran","year":"2004","journal-title":"Eur. J. Biochem."},{"key":"2023072122511031800_bib27","doi-asserted-by":"publisher","first-page":"1462","DOI":"10.1021\/tx700108w","article-title":"Oxidation and inactivation of SERCA by selective reaction of cysteine residues with amino acid peroxides","volume":"20","author":"Dremina","year":"2007","journal-title":"Chem. Res. Toxicol."},{"key":"2023072122511031800_bib28","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1016\/s0959-437x(02)00309-x","article-title":"Muscular dystrophies involving the dystrophin-glycoprotein complex: An overview of current mouse models","volume":"12","author":"Durbeej","year":"2002","journal-title":"Curr. Opin. Genet. Dev."},{"key":"2023072122511031800_bib29","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1016\/j.cell.2008.02.042","article-title":"RyR1 S-Nitrosylation underlies environmental heat stroke and sudden death in Y522S RyR1 knockin mice","volume":"133","author":"Durham","year":"2008","journal-title":"Cell"},{"key":"2023072122511031800_bib30","doi-asserted-by":"publisher","first-page":"C42","DOI":"10.1152\/ajpcell.00524.2009","article-title":"Upregulation of store-operated Ca2+ entry in dystrophic mdx mouse muscle","volume":"299","author":"Edwards","year":"2010","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"2023072122511031800_bib31","doi-asserted-by":"publisher","first-page":"609","DOI":"10.1002\/mus.21154","article-title":"Sarcoplasmic-endoplasmic-reticulum Ca2+-ATPase and calsequestrin are overexpressed in spared intrinsic laryngeal muscles of dystrophin-deficient mdx mice","volume":"39","author":"Ferretti","year":"2009","journal-title":"Muscle Nerve"},{"key":"2023072122511031800_bib32","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1038\/nature04702","article-title":"A mutation in orai1 causes immune deficiency by abrogating CRAC channel function","volume":"441","author":"Feske","year":"2006","journal-title":"Nature"},{"key":"2023072122511031800_bib33","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1016\/s0960-8966(01)00220-6","article-title":"Non-toxic ubiquitous over-expression of utrophin in the mdx mouse","volume":"11","author":"Fisher","year":"2001","journal-title":"Neuromuscul. Disord."},{"key":"2023072122511031800_bib34","doi-asserted-by":"publisher","first-page":"363","DOI":"10.3389\/fphys.2013.00363","article-title":"Nuclear positioning in muscle development and disease","volume":"4","author":"Folker","year":"2013","journal-title":"Front. Physiol."},{"key":"2023072122511031800_bib35","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1126\/science.2173137","article-title":"Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin","volume":"250","author":"Fong","year":"1990","journal-title":"Science"},{"key":"2023072122511031800_bib36","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1113\/jphysiol.1990.sp018176","article-title":"Stretch-sensitive channels in developing muscle cells from a mouse cell line","volume":"427","author":"Franco","year":"1990","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib37","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1113\/jphysiol.1992.sp019125","article-title":"A caffeine- and ryanodine-sensitive Ca2+ store in bullfrog sympathetic neurones modulates effects of Ca2+ entry on [Ca2+]i","volume":"450","author":"Friel","year":"1992","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib38","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1085\/jgp.103.1.107","article-title":"Measurement of calcium transients and slow calcium current in myotubes","volume":"103","author":"Garc\u00eda","year":"1994","journal-title":"J. Gen. Physiol."},{"key":"2023072122511031800_bib39","doi-asserted-by":"publisher","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":"2023072122511031800_bib40","doi-asserted-by":"publisher","first-page":"3706","DOI":"10.1093\/hmg\/ddu079","article-title":"Enhanced Ca2+ influx from STIM1-Orai1 induces muscle pathology in mouse models of muscular dystrophy","volume":"23","author":"Goonasekera","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"2023072122511031800_bib41","doi-asserted-by":"publisher","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":"2023072122511031800_bib42","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1007\/978-1-61737-982-6_5","article-title":"Monitoring murine skeletal muscle function for muscle gene therapy","volume":"709","author":"Hakim","year":"2011","journal-title":"Methods Mol. Biol."},{"key":"2023072122511031800_bib43","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1113\/expphysiol.2009.052019","article-title":"Branched fibres in old dystrophic mdx muscle are associated with mechanical weakening of the sarcolemma, abnormal Ca2+ transients and a breakdown of Ca2+ homeostasis during fatigue","volume":"95","author":"Head","year":"2010","journal-title":"Exp. Physiol."},{"key":"2023072122511031800_bib44","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1016\/0960-8966(92)90004-p","article-title":"The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathies","volume":"2","author":"Helliwell","year":"1992","journal-title":"Neuromuscul. Disord."},{"key":"2023072122511031800_bib45","doi-asserted-by":"publisher","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":"2023072122511031800_bib46","doi-asserted-by":"publisher","first-page":"5063","DOI":"10.1113\/jphysiol.2008.160507","article-title":"Comparison of the myoplasmic calcium transient elicited by an action potential in intact fibres of mdx and normal mice","volume":"586","author":"Hollingworth","year":"2008","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib47","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1038\/355696A0","article-title":"Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix","volume":"355","author":"Ibraghimov-Beskrovnaya","year":"1992","journal-title":"Nature"},{"key":"2023072122511031800_bib48","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1016\/0304-4165(95)00180-8","article-title":"The sarcoplasmic reticulum calcium pump is functionally altered in dystrophic muscle","volume":"1290","author":"Kargacin","year":"1996","journal-title":"Biochim. Biophys. Acta"},{"key":"2023072122511031800_bib49","doi-asserted-by":"publisher","first-page":"244","DOI":"10.1038\/nm.2598","article-title":"AICAR prevents heat-induced sudden death in RyR1 mutant mice independent of AMPK activation","volume":"18","author":"Lanner","year":"2012","journal-title":"Nat. Med."},{"key":"2023072122511031800_bib50","doi-asserted-by":"publisher","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":"2023072122511031800_bib51","doi-asserted-by":"publisher","first-page":"E228","DOI":"10.1073\/pnas.1619238114","article-title":"Congenital myopathy results from misregulation of a muscle Ca2+ channel by mutant Stac3","volume":"114","author":"Linsley","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072122511031800_bib52","doi-asserted-by":"publisher","first-page":"1235","DOI":"10.1016\/j.cub.2005.05.055","article-title":"STIM is a Ca2+ sensor essential for Ca2+-store-depletion-triggered Ca2+ influx","volume":"15","author":"Liou","year":"2005","journal-title":"Curr. Biol."},{"key":"2023072122511031800_bib53","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.ymthe.2004.09.013","article-title":"Adeno-associated virus-mediated microdystrophin expression protects young mdx muscle from contraction-induced injury","volume":"11","author":"Liu","year":"2005","journal-title":"Mol. Ther."},{"key":"2023072122511031800_bib54","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1093\/oxfordjournals.bmb.a072351","article-title":"Molecular analysis of Duchenne and Becker muscular dystrophies","volume":"45","author":"Love","year":"1989","journal-title":"Br. Med. Bull."},{"key":"2023072122511031800_bib55","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1085\/jgp.201010523","article-title":"Muscle weakness in Ryr1I4895T\/WT knock-in mice as a result of reduced ryanodine receptor Ca2+ ion permeation and release from the sarcoplasmic reticulum","volume":"137","author":"Loy","year":"2011","journal-title":"J. Gen. Physiol."},{"key":"2023072122511031800_bib56","doi-asserted-by":"publisher","first-page":"4815","DOI":"10.1113\/jphysiol.2008.160481","article-title":"Differential dependence of store-operated and excitation-coupled Ca2+ entry in skeletal muscle on STIM1 and orai1","volume":"586","author":"Lyfenko","year":"2008","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib57","doi-asserted-by":"publisher","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":"2023072122511031800_bib58","doi-asserted-by":"publisher","first-page":"4950","DOI":"10.1073\/pnas.97.9.4950","article-title":"Elevated subsarcolemmal Ca2+ in mdx mouse skeletal muscle fibers detected with Ca2+-activated K+ channels","volume":"97","author":"Mallouk","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072122511031800_bib59","doi-asserted-by":"publisher","first-page":"647010","DOI":"10.3389\/fphys.2021.647010","article-title":"Abnormal calcium handling in duchenne muscular dystrophy: Mechanisms and potential therapies","volume":"12","author":"Mareedu","year":"2021","journal-title":"Front. Physiol."},{"key":"2023072122511031800_bib60","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0065831","article-title":"Isobaric tagging-based quantification for proteomic analysis: A comparative study of spared and affected muscles from mdx mice at the early phase of dystrophy","volume":"8","author":"Matsumura","year":"2013","journal-title":"PLoS One"},{"key":"2023072122511031800_bib61","doi-asserted-by":"publisher","first-page":"6936897","DOI":"10.1155\/2017\/6936897","article-title":"Estrogens protect calsequestrin-1 knockout mice from lethal hyperthermic episodes by reducing oxidative stress in muscle","volume":"2017","author":"Michelucci","year":"2017","journal-title":"Oxid. Med. Cell. Longev."},{"key":"2023072122511031800_bib62","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.47576","article-title":"Transverse tubule remodeling enhances orai1-dependent Ca2+ entry in skeletal muscle","volume":"8","author":"Michelucci","year":"2019","journal-title":"Elife"},{"key":"2023072122511031800_bib63","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.202012617","article-title":"Pre-assembled Ca2+ entry units and constitutively active Ca2+ entry in skeletal muscle of calsequestrin-1 knockout mice","volume":"152","author":"Michelucci","year":"2020","journal-title":"J. Gen. Physiol."},{"key":"2023072122511031800_bib64","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.ceca.2018.10.004","article-title":"Role of STIM1\/ORAI1-mediated store-operated Ca2+ entry in skeletal muscle physiology and disease","volume":"76","author":"Michelucci","year":"2018","journal-title":"Cell Calcium"},{"key":"2023072122511031800_bib65","doi-asserted-by":"publisher","first-page":"424","DOI":"10.3390\/metabo11070424","article-title":"Altered Ca2+ handling and oxidative stress underlie mitochondrial damage and skeletal muscle dysfunction in aging and disease","volume":"11","author":"Michelucci","year":"2021","journal-title":"Metabolites"},{"key":"2023072122511031800_bib66","doi-asserted-by":"publisher","first-page":"6792694","DOI":"10.1155\/2017\/6792694","article-title":"Antioxidant treatment reduces formation of structural cores and improves muscle function in RYR1Y522S\/WT mice","volume":"2017","author":"Michelucci","year":"2017","journal-title":"Oxid. Med. Cell. Longev."},{"key":"2023072122511031800_bib67","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1097\/ALN.0000000000000748","article-title":"Antioxidants protect calsequestrin-1 knockout mice from halothane- and heat-induced sudden death","volume":"123","author":"Michelucci","year":"2015","journal-title":"Anesthesiology"},{"key":"2023072122511031800_bib68","doi-asserted-by":"publisher","first-page":"19023","DOI":"10.1073\/pnas.0906591106","article-title":"Calcium influx is sufficient to induce muscular dystrophy through a TRPC-dependent mechanism","volume":"106","author":"Millay","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072122511031800_bib69","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1038\/323646A0","article-title":"Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy gene","volume":"323","author":"Monaco","year":"1986","journal-title":"Nature"},{"key":"2023072122511031800_bib70","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1126\/science.aad4076","article-title":"A peptide encoded by a transcript annotated as long noncoding RNA enhances SERCA activity in muscle","volume":"351","author":"Nelson","year":"2016","journal-title":"Science"},{"key":"2023072122511031800_bib71","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1083\/jcb.112.1.135","article-title":"Dystrophin-glycoprotein complex is highly enriched in isolated skeletal muscle sarcolemma","volume":"112","author":"Ohlendieck","year":"1991","journal-title":"J. Cell Biol."},{"key":"2023072122511031800_bib72","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.abb.2015.01.025","article-title":"Store-operated calcium entry contributes to abnormal Ca2+ signalling in dystrophic mdx mouse myoblasts","volume":"569","author":"Onopiuk","year":"2015","journal-title":"Arch. Biochem. Biophys."},{"key":"2023072122511031800_bib73","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1111\/j.1365-2613.2009.00688.x","article-title":"Calcium-binding proteins in skeletal muscles of the mdx mice: Potential role in the pathogenesis of duchenne muscular dystrophy","volume":"91","author":"Pertille","year":"2010","journal-title":"Int. J. Exp. Pathol."},{"key":"2023072122511031800_bib74","doi-asserted-by":"publisher","first-page":"2781","DOI":"10.1093\/hmg\/ddx173","article-title":"Oxidative stress in duchenne muscular dystrophy: Focus on the NRF2 redox pathway","volume":"26","author":"Petrillo","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"2023072122511031800_bib75","doi-asserted-by":"publisher","first-page":"119296","DOI":"10.1016\/j.lfs.2021.119296","article-title":"Reduced sarcoplasmic reticulum Ca2+ ATPase activity underlies skeletal muscle wasting in asthma","volume":"273","author":"Qaisar","year":"2021","journal-title":"Life Sci."},{"key":"2023072122511031800_bib76","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1007\/S12576-009-0069-Z","article-title":"The cell boundary theorem: A simple law of the control of cytosolic calcium concentration","volume":"60","author":"R\u00edos","year":"2010","journal-title":"J. Physiol. Sci."},{"key":"2023072122511031800_bib77","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1083\/jcb.200502019","article-title":"STIM1, an essential and conserved component of store-operated Ca2+ channel function","volume":"169","author":"Roos","year":"2005","journal-title":"J. Cell Biol."},{"key":"2023072122511031800_bib78","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1002\/mus.20512","article-title":"Sarcoplasmic reticulum: The dynamic calcium governor of muscle","volume":"33","author":"Rossi","year":"2006","journal-title":"Muscle Nerve"},{"key":"2023072122511031800_bib79","doi-asserted-by":"publisher","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":"2023072122511031800_bib80","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1042\/BJ20051214","article-title":"Quantitative mapping of oxidation-sensitive cysteine residues in SERCA in vivo and in vitro by HPLC-electrospray-tandem MS: Selective protein oxidation during biological aging","volume":"394","author":"Sharov","year":"2006","journal-title":"Biochem. J."},{"key":"2023072122511031800_bib81","doi-asserted-by":"publisher","first-page":"10909","DOI":"10.1074\/jbc.270.18.10909","article-title":"Calpains are activated in necrotic fibers from mdx dystrophic mice","volume":"270","author":"Spencer","year":"1995","journal-title":"J. Biol. Chem."},{"key":"2023072122511031800_bib82","doi-asserted-by":"publisher","first-page":"2645","DOI":"10.1093\/hmg\/11.21.2645","article-title":"Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology","volume":"11","author":"Spencer","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"2023072122511031800_bib83","doi-asserted-by":"publisher","first-page":"688","DOI":"10.1038\/ncb1731","article-title":"STIM1 signalling controls store-operated calcium entry required for development and contractile function in skeletal muscle","volume":"10","author":"Stiber","year":"2008","journal-title":"Nat. Cell Biol."},{"key":"2023072122511031800_bib84","doi-asserted-by":"publisher","first-page":"2496","DOI":"10.1016\/j.bpj.2017.09.023","article-title":"SOCE is important for maintaining sarcoplasmic calcium content and release in skeletal muscle fibers","volume":"113","author":"Sztretye","year":"2017","journal-title":"Biophys. J."},{"key":"2023072122511031800_bib85","doi-asserted-by":"publisher","first-page":"819","DOI":"10.1113\/jphysiol.2002.024935","article-title":"Expression of a calpastatin transgene slows muscle wasting and obviates changes in myosin isoform expression during murine muscle disuse","volume":"545","author":"Tidball","year":"2002","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib86","doi-asserted-by":"publisher","first-page":"1441","DOI":"10.1038\/4033","article-title":"Expression of full-length utrophin prevents muscular dystrophy in mdx mice","volume":"4","author":"Tinsley","year":"1998","journal-title":"Nat. Med."},{"key":"2023072122511031800_bib87","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1038\/360591A0","article-title":"Primary structure of dystrophin-related protein","volume":"360","author":"Tinsley","year":"1992","journal-title":"Nature"},{"key":"2023072122511031800_bib88","doi-asserted-by":"publisher","first-page":"56024","DOI":"10.3791\/56024","article-title":"Semi-automated analysis of mouse skeletal muscle morphology and fiber-type composition","author":"Tyagi","year":"2017","journal-title":"J. Vis. Exp."},{"key":"2023072122511031800_bib89","doi-asserted-by":"publisher","first-page":"1089","DOI":"10.1083\/jcb.200203091","article-title":"Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers","volume":"158","author":"Vandebrouck","year":"2002","journal-title":"J. Cell Biol."},{"key":"2023072122511031800_bib90","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1038\/s41582-019-0203-3","article-title":"Therapeutic developments for Duchenne muscular dystrophy","volume":"15","author":"Verhaart","year":"2019","journal-title":"Nat. Rev. Neurol."},{"key":"2023072122511031800_bib91","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1126\/science.1127883","article-title":"CRACM1 is a plasma membrane protein essential for store-operated Ca2+ entry","volume":"312","author":"Vig","year":"2006","journal-title":"Science"},{"key":"2023072122511031800_bib92","doi-asserted-by":"publisher","first-page":"243","DOI":"10.3109\/10715769609088022","article-title":"The oxidative inactivation of sarcoplasmic reticulum Ca(2+)-ATPase by peroxynitrite","volume":"24","author":"Viner","year":"1996","journal-title":"Free Radic. Res."},{"key":"2023072122511031800_bib93","doi-asserted-by":"publisher","first-page":"2805","DOI":"10.1038\/ncomms3805","article-title":"Orai1-dependent calcium entry promotes skeletal muscle growth and limits fatigue","volume":"4","author":"Wei-Lapierre","year":"2013","journal-title":"Nat. Commun."},{"key":"2023072122511031800_bib94","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.202112976","article-title":"Acute exposure to extracellular BTP2 does not inhibit Ca2+ release during EC coupling in intact skeletal muscle fibers","volume":"154","author":"Wei-LaPierre","year":"2022","journal-title":"J. Gen. Physiol."},{"key":"2023072122511031800_bib95","doi-asserted-by":"publisher","first-page":"2003","DOI":"10.1113\/jphysiol.2007.148338","article-title":"N-Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice","volume":"586","author":"Whitehead","year":"2008","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib96","doi-asserted-by":"publisher","first-page":"867","DOI":"10.1113\/jphysiol.2005.089318","article-title":"Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres","volume":"568","author":"Woods","year":"2005","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib97","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1113\/jphysiol.2004.061291","article-title":"The action potential-evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres","volume":"557","author":"Woods","year":"2004","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib98","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1038\/nature05108","article-title":"Molecular identification of the CRAC channel by altered ion selectivity in a mutant of orai","volume":"443","author":"Yeromin","year":"2006","journal-title":"Nature"},{"key":"2023072122511031800_bib99","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1113\/jphysiol.2004.075275","article-title":"Effects of stretch-activated channel blockers on [Ca2+]i and muscle damage in the mdx mouse","volume":"562","author":"Yeung","year":"2005","journal-title":"J. Physiol."},{"key":"2023072122511031800_bib100","doi-asserted-by":"publisher","first-page":"11040","DOI":"10.3390\/ijms222011040","article-title":"Disrupted calcium homeostasis in duchenne muscular dystrophy: A common mechanism behind diverse consequences","volume":"22","author":"Zab\u0142ocka","year":"2021","journal-title":"Int. J. Mol. Sci."},{"key":"2023072122511031800_bib101","doi-asserted-by":"publisher","first-page":"902","DOI":"10.1038\/nature04147","article-title":"STIM1 is a Ca2+ sensor that activates CRAC channels and migrates from the Ca2+ store to the plasma membrane","volume":"437","author":"Zhang","year":"2005","journal-title":"Nature"},{"key":"2023072122511031800_bib102","doi-asserted-by":"publisher","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":"2023072122511031800_bib103","doi-asserted-by":"publisher","first-page":"12427","DOI":"10.1074\/jbc.M309297200","article-title":"Potent inhibition of Ca2+ release-activated Ca2+ channels and T-lymphocyte activation by the pyrazole derivative BTP2","volume":"279","author":"Zitt","year":"2004","journal-title":"J. Biol. Chem."},{"key":"2023072122511031800_bib104","doi-asserted-by":"publisher","first-page":"18537","DOI":"10.1073\/pnas.0709312104","article-title":"An Ryr1I4895T mutation abolishes Ca2+ release channel function and delays development in homozygous offspring of a mutant mouse line","volume":"104","author":"Zvaritch","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"}],"container-title":["Journal of General Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/rupress.org\/jgp\/article-pdf\/doi\/10.1085\/jgp.202213081\/1456983\/jgp_202213081.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/rupress.org\/jgp\/article-pdf\/doi\/10.1085\/jgp.202213081\/1456983\/jgp_202213081.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,21]],"date-time":"2023-07-21T18:54:25Z","timestamp":1689965665000},"score":1,"resource":{"primary":{"URL":"https:\/\/rupress.org\/jgp\/article\/154\/9\/e202213081\/213383\/Postdevelopmental-knockout-of-Orai1-improves"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,8]]},"references-count":105,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2022,9,5]]}},"URL":"https:\/\/doi.org\/10.1085\/jgp.202213081","relation":{},"ISSN":["0022-1295","1540-7748"],"issn-type":[{"value":"0022-1295","type":"print"},{"value":"1540-7748","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,8]]},"article-number":"e202213081"}}