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Among these, recessive loss\u2010of\u2010function\n                    <jats:italic>MYH3<\/jats:italic>\n                    mutations lead to spondylocarpotarsal synostosis (SCTS), characterized by vertebral fusions and scoliosis. We find that\n                    <jats:italic>Myh3<\/jats:italic>\n                    germline knockout adult mice display SCTS phenotypes such as scoliosis and vertebral fusion, in addition to reduced body weight, muscle weight, myofiber size, and grip strength.\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockout mice also exhibit changes in muscle fiber type, altered satellite cell numbers and increased muscle fibrosis. A mass spectrometric analysis of embryonic skeletal muscle from\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockouts identified integrin signaling and cytoskeletal regulation as the most affected pathways. These pathways are closely connected to the mechanosensing Yes\u2010associated protein (YAP) transcriptional regulator, which we found to be significantly activated in the skeletal muscle of\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockout mice. To test whether increased YAP signaling might underlie the musculoskeletal defects in\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockout mice, we treated these mice with CA3, a small molecule inhibitor of YAP signaling. This led to increased muscle fiber size, rescue of most muscle fiber type alterations, normalization of the satellite cell marker Pax7 levels, increased grip strength, reduced fibrosis, and decline in scoliosis in\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockout mice. Thus, increased YAP activation underlies the musculoskeletal defects seen in\n                    <jats:italic>Myh3<\/jats:italic>\n                    knockout mice, indicating its significance as a key pathway to target in SCTS and other\n                    <jats:italic>MYH3<\/jats:italic>\n                    \u2010related congenital syndromes.\n                  <\/jats:p>","DOI":"10.15252\/emmm.202217187","type":"journal-article","created":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T03:46:23Z","timestamp":1690343183000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Musculoskeletal defects associated with myosin heavy chain\u2010embryonic loss of function are mediated by the YAP signaling pathway"],"prefix":"10.1038","volume":"15","author":[{"given":"Anushree","family":"Bharadwaj","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaydeep","family":"Sharma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jagriti","family":"Singh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahima","family":"Kumari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5996-7115","authenticated-orcid":false,"given":"Tanushri","family":"Dargar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7406-5689","authenticated-orcid":false,"given":"Bhargab","family":"Kalita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7908-6094","authenticated-orcid":false,"given":"Sam J","family":"Mathew","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,7,26]]},"reference":[{"key":"BFEMMM202217187_CR1","doi-asserted-by":"crossref","DOI":"10.1242\/dev.184507","volume":"147","author":"M Agarwal","year":"2020","unstructured":"Agarwal M, Sharma A, Kumar P, Kumar A, Bharadwaj A, Saini M, Kardon G, Mathew SJ (2020) Myosin heavy chain\u2010embryonic regulates skeletal muscle differentiation during mammalian development. 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