{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T21:47:31Z","timestamp":1790372851331,"version":"4.1.0"},"reference-count":71,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T00:00:00Z","timestamp":1708560000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Cure CMD","award":["2022-2024"],"award-info":[{"award-number":["2022-2024"]}]},{"name":"Cure CMD","award":["2018-2020"],"award-info":[{"award-number":["2018-2020"]}]},{"name":"Cure CMD","award":["PRIN2017"],"award-info":[{"award-number":["PRIN2017"]}]},{"name":"Cure CMD","award":["201742SBXA"],"award-info":[{"award-number":["201742SBXA"]}]},{"name":"Cure CMD","award":["2022-2024"],"award-info":[{"award-number":["2022-2024"]}]},{"name":"Cure CMD","award":["2018-2020"],"award-info":[{"award-number":["2018-2020"]}]},{"name":"Cure CMD","award":["PRIN2017"],"award-info":[{"award-number":["PRIN2017"]}]},{"name":"Cure CMD","award":["201742SBXA"],"award-info":[{"award-number":["201742SBXA"]}]},{"DOI":"10.13039\/501100003407","name":"Italian MIUR","doi-asserted-by":"publisher","award":["2022-2024"],"award-info":[{"award-number":["2022-2024"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"Italian MIUR","doi-asserted-by":"publisher","award":["2018-2020"],"award-info":[{"award-number":["2018-2020"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"Italian MIUR","doi-asserted-by":"publisher","award":["PRIN2017"],"award-info":[{"award-number":["PRIN2017"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"Italian MIUR","doi-asserted-by":"publisher","award":["201742SBXA"],"award-info":[{"award-number":["201742SBXA"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>The pericellular matrix (PCM) is a specialized extracellular matrix that surrounds cells. Interactions with the PCM enable the cells to sense and respond to mechanical signals, triggering a proper adaptive response. Collagen VI is a component of muscle and tendon PCM. Mutations in collagen VI genes cause a distinctive group of inherited skeletal muscle diseases, and Ullrich congenital muscular dystrophy (UCMD) is the most severe form. In addition to muscle weakness, UCMD patients show structural and functional changes of the tendon PCM. In this study, we investigated whether PCM alterations due to collagen VI mutations affect the response of tendon fibroblasts to mechanical stimulation. By taking advantage of human tendon cultures obtained from unaffected donors and from UCMD patients, we analyzed the morphological and functional properties of cellular mechanosensors. We found that the length of the primary cilia of UCMD cells was longer than that of controls. Unlike controls, in UCMD cells, both cilia prevalence and length were not recovered after mechanical stimulation. Accordingly, under the same experimental conditions, the activation of the Hedgehog signaling pathway, which is related to cilia activity, was impaired in UCMD cells. Finally, UCMD tendon cells exposed to mechanical stimuli showed altered focal adhesions, as well as impaired activation of Akt, ERK1\/2, p38MAPK, and mechanoresponsive genes downstream of YAP. By exploring the response to mechanical stimulation, for the first time, our findings uncover novel unreported mechanistic aspects of the physiopathology of UCMD-derived tendon fibroblasts and point at a role for collagen VI in the modulation of mechanotransduction in tendons.<\/jats:p>","DOI":"10.3390\/cells13050378","type":"journal-article","created":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T08:34:35Z","timestamp":1708590875000},"page":"378","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Collagen VI Deficiency Impairs Tendon Fibroblasts Mechanoresponse in Ullrich Congenital Muscular Dystrophy"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8062-1840","authenticated-orcid":false,"given":"Vittoria","family":"Cenni","sequence":"first","affiliation":[{"name":"CNR-Institute of Molecular Genetics, via di Barbiano 1\/10, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrizia","family":"Sabatelli","sequence":"additional","affiliation":[{"name":"CNR-Institute of Molecular Genetics, via di Barbiano 1\/10, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0716-4951","authenticated-orcid":false,"given":"Alberto","family":"Di Martino","sequence":"additional","affiliation":[{"name":"1st Orthopedics and Traumatology Department, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"},{"name":"Department of Biomedical and Neuromotor Science, DIBINEM, University of Bologna, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luciano","family":"Merlini","sequence":"additional","affiliation":[{"name":"Department of Biomedical and Neuromotor Science, DIBINEM, University of Bologna, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Antoniel","sequence":"additional","affiliation":[{"name":"CNR-Institute of Molecular Genetics, via di Barbiano 1\/10, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6538-5923","authenticated-orcid":false,"given":"Stefano","family":"Squarzoni","sequence":"additional","affiliation":[{"name":"CNR-Institute of Molecular Genetics, via di Barbiano 1\/10, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3257-6293","authenticated-orcid":false,"given":"Simona","family":"Neri","sequence":"additional","affiliation":[{"name":"Medicine and Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9856-7053","authenticated-orcid":false,"given":"Spartaco","family":"Santi","sequence":"additional","affiliation":[{"name":"CNR-Institute of Molecular Genetics, via di Barbiano 1\/10, 40136 Bologna, Italy"},{"name":"IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuele","family":"Metti","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Padova, 35122 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9571-8140","authenticated-orcid":false,"given":"Paolo","family":"Bonaldo","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine, University of Padova, 35122 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cesare","family":"Faldini","sequence":"additional","affiliation":[{"name":"1st Orthopedics and Traumatology Department, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy"},{"name":"Department of Biomedical and Neuromotor Science, DIBINEM, University of Bologna, 40136 Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,22]]},"reference":[{"key":"ref_1","first-page":"3525","article-title":"Collagen VI at a glance","volume":"128","author":"Cescon","year":"2015","journal-title":"J. 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