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To date, no specific therapy is available to treat muscle weakness in nemaline myopathy. We tested the ability of tirasemtiv, a fast skeletal troponin activator that targets the thin filament, to augment muscle force\u2014both in vivo and in vitro\u2014in a nemaline myopathy mouse model with a mutation (H40Y) in Acta1. In Acta1H40Y mice, treatment with tirasemtiv increased the force response of muscles to submaximal stimulation frequencies. This resulted in a reduced energetic cost of force generation, which increases the force production during a fatigue protocol. The inotropic effects of tirasemtiv were present in locomotor muscles and, albeit to a lesser extent, in respiratory muscles, and they persisted during chronic treatment, an important finding as respiratory failure is the main cause of death in patients with congenital myopathy. Finally, translational studies on permeabilized muscle fibers isolated from a biopsy of a patient with the ACTA1H40Y mutation revealed that at physiological Ca2+ concentrations, tirasemtiv increased force generation to values that were close to those generated in muscle fibers of healthy subjects. These findings indicate the therapeutic potential of fast skeletal muscle troponin activators to improve muscle function in nemaline myopathy due to the ACTA1H40Y mutation, and future studies should assess their merit for other forms of nemaline myopathy and for other congenital myopathies.<\/jats:p>","DOI":"10.1093\/hmg\/ddab112","type":"journal-article","created":{"date-parts":[[2021,4,23]],"date-time":"2021-04-23T19:58:46Z","timestamp":1619207926000},"page":"1305-1320","source":"Crossref","is-referenced-by-count":22,"title":["Acute and chronic <i>tirasemtiv<\/i> treatment improves <i>in vivo<\/i> and <i>in vitro<\/i> muscle performance in actin-based nemaline myopathy mice"],"prefix":"10.1093","volume":"30","author":[{"given":"Josine M","family":"de Winter","sequence":"first","affiliation":[{"name":"Department of Physiology, Amsterdam UMC (location VUmc), Amsterdam 1081 HV, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Gineste","sequence":"additional","affiliation":[{"name":"Aix-Marseille Univ, CNRS, CRMBM, UMR 7339, 13005 Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Minardi","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenza","family":"Brocca","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maira","family":"Rossi","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamara","family":"Borsboom","sequence":"additional","affiliation":[{"name":"Department of Physiology, Amsterdam UMC (location VUmc), Amsterdam 1081 HV, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan H","family":"Beggs","sequence":"additional","affiliation":[{"name":"Division of Genetics and Genomics, The Manton Center for Orphan Disease Research, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique","family":"Bernard","sequence":"additional","affiliation":[{"name":"Aix-Marseille Univ, CNRS, CRMBM, UMR 7339, 13005 Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Bendahan","sequence":"additional","affiliation":[{"name":"Aix-Marseille Univ, CNRS, CRMBM, UMR 7339, 13005 Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darren T","family":"Hwee","sequence":"additional","affiliation":[{"name":"Research and Early Development, Cytokinetics Inc., South San Francisco, CA 94080, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fady I","family":"Malik","sequence":"additional","affiliation":[{"name":"Research and Early Development, Cytokinetics Inc., South San Francisco, CA 94080, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Antonietta","family":"Pellegrino","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy"},{"name":"Interdipartimental Centre for Biology and Sport Medicine, University of Pavia, Pavia 27100, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Bottinelli","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy"},{"name":"Istituti Clinici Maugeri (IRCCS), Scientific Institute of Pavia, Pavia 27100, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Gondin","sequence":"additional","affiliation":[{"name":"Aix-Marseille Univ, CNRS, CRMBM, UMR 7339, 13005 Marseille, France"},{"name":"Institut NeuroMyoG\u00e8ne, Universit\u00e9 Claude Bernard Lyon 1, CNRS 5310, INSERM U1217, 69008, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Coen A C","family":"Ottenheijm","sequence":"additional","affiliation":[{"name":"Department of Physiology, Amsterdam UMC (location VUmc), Amsterdam 1081 HV, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,4,28]]},"reference":[{"key":"2021070819130491400_ref1","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1038\/nrneurol.2017.191","article-title":"Congenital myopathies: disorders of excitation-contraction coupling and muscle contraction. 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