{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"institution":[{"name":"bioRxiv"}],"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T01:40:33Z","timestamp":1772156433090,"version":"3.50.1"},"posted":{"date-parts":[[2024,11,25]]},"group-title":"Developmental Biology","reference-count":48,"publisher":"openRxiv","license":[{"start":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T00:00:00Z","timestamp":1732492800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"accepted":{"date-parts":[[2024,11,25]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                <jats:p>\n                  Mutations in protein\n                  <jats:italic>O<\/jats:italic>\n                  -glucosyltransferase 1 (\n                  <jats:italic>POGLUT1<\/jats:italic>\n                  ) cause a recessive form of limb-girdle muscular dystrophy (LGMD-R21) associated with reduced satellite cell number and NOTCH1 signaling in adult patient muscles and impaired myogenic capacity of patient-derived muscle progenitors. However, the\n                  <jats:italic>in vivo<\/jats:italic>\n                  roles of POGLUT1 in the development, function, and maintenance of satellite cells are not well understood. Here, we show that conditional deletion of mouse\n                  <jats:italic>Poglut1<\/jats:italic>\n                  in myogenic progenitors leads to early lethality, postnatal muscle growth defects, reduced\n                  <jats:italic>Pax7<\/jats:italic>\n                  expression, abnormality in muscle extracellular matrix, and impaired muscle repair.\n                  <jats:italic>Poglut1<\/jats:italic>\n                  -deficient muscle progenitors exhibit reduced proliferation, enhanced differentiation, and accelerated fusion into myofibers. Inducible loss of\n                  <jats:italic>Poglut1<\/jats:italic>\n                  in adult satellite cells leads to their precocious differentiation and impairs muscle repair upon serial injury. Cell-based signaling assays and mass spectrometric analysis indicate that POGLUT1 is required for the activation of NOTCH1, NOTCH2, and NOTCH3 in myoblasts and that NOTCH3 is a target of POGLUT1 like NOTCH1 and NOTCH2. These observations provide insight into the roles of POGLUT1 in muscle development and repair and the pathophysiology of LGMD-R21.\n                <\/jats:p>","DOI":"10.1101\/2024.11.25.625261","type":"posted-content","created":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T22:51:37Z","timestamp":1732575097000},"source":"Crossref","is-referenced-by-count":0,"title":["The glycosyltransferase POGLUT1 regulates muscle stem cell development and maintenance in mice"],"prefix":"10.64898","author":[{"given":"Soomin","family":"Cho","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emilia","family":"Servi\u00e1n-Morilla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Victoria Navarro","family":"Garrido","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatriz","family":"Rodriguez-Gonzalez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Youxi","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raquel","family":"Cano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arjun A.","family":"Rambhiya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Radbod","family":"Darabi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert 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