{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:59:03Z","timestamp":1791896343933,"version":"4.3.4"},"reference-count":89,"publisher":"American Society for Clinical Investigation","issue":"10","license":[{"start":{"date-parts":[[2022,5,23]],"date-time":"2022-05-23T00:00:00Z","timestamp":1653264000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"the Samsung Science and Technology Foundation","award":["SSTF-BA1702-11"],"award-info":[{"award-number":["SSTF-BA1702-11"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,5,23]]},"abstract":"<jats:p>\n                    The survival of motor neuron (SMN) protein is a major component of the pre-mRNA splicing machinery and is required for RNA metabolism. Although\n                    <jats:italic>SMN<\/jats:italic>\n                    has been considered a fundamental gene for the central nervous system, due to its relationship with neuromuscular diseases, such as spinal muscular atrophy, recent studies have also revealed the requirement of SMN in non-neuronal cells in the peripheral regions. Here, we report that the fibro-adipogenic progenitor subpopulation expressing Dpp4 (Dpp4\n                    <jats:sup>+<\/jats:sup>\n                    FAPs) is required for the neuromuscular system. Furthermore, we also reveal that BRCA1-associated protein-1 (Bap1) is crucial for the stabilization of SMN in FAPs by preventing its ubiquitination-dependent degradation. Inactivation of\n                    <jats:italic>Bap1<\/jats:italic>\n                    in FAPs decreased SMN levels and accompanied degeneration of the neuromuscular junction, leading to loss of motor neurons and muscle atrophy. Overexpression of the ubiquitination-resistant SMN variant,\n                    <jats:italic>\n                      SMN\n                      <jats:sup>K186R<\/jats:sup>\n                    <\/jats:italic>\n                    , in\n                    <jats:italic>Bap1<\/jats:italic>\n                    -null FAPs completely prevented neuromuscular degeneration. In addition, transplantation of Dpp4\n                    <jats:sup>+<\/jats:sup>\n                    FAPs, but not Dpp4\n                    <jats:sup>\u2013<\/jats:sup>\n                    FAPs, completely rescued neuromuscular defects. Our data reveal the crucial role of\n                    <jats:italic>Bap1<\/jats:italic>\n                    -mediated SMN stabilization in Dpp4\n                    <jats:sup>+<\/jats:sup>\n                    FAPs for the neuromuscular system and provide the possibility of cell-based therapeutics to treat neuromuscular diseases.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.158380","type":"journal-article","created":{"date-parts":[[2022,5,22]],"date-time":"2022-05-22T14:00:55Z","timestamp":1653228055000},"source":"Crossref","is-referenced-by-count":28,"title":["Bap1\/SMN axis in Dpp4+ skeletal muscle mesenchymal cells regulates the neuromuscular system"],"prefix":"10.1172","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9217-4483","authenticated-orcid":true,"given":"Ji-Hoon","family":"Kim","sequence":"first","affiliation":[{"name":"School of Biological Sciences, Seoul National University, Seoul, South Korea."},{"name":"Molecular Recognition Research 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