{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T12:55:22Z","timestamp":1781614522442,"version":"3.54.5"},"reference-count":69,"publisher":"American Society for Clinical Investigation","issue":"9","license":[{"start":{"date-parts":[[2021,5,10]],"date-time":"2021-05-10T00:00:00Z","timestamp":1620604800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institute of Health","award":["R01GM098309,NIHT32DK007203"],"award-info":[{"award-number":["R01GM098309,NIHT32DK007203"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,5,10]]},"abstract":"<jats:p>\n                    Activating mutations in the fibroblast growth factor receptor 3 (FGFR3) or inactivating mutations in guanylyl cyclase\u2013B (GC-B), also known as NPR-B or Npr2, cause short-limbed dwarfism. FGFR3 activation causes dephosphorylation and inactivation of GC-B, but the contribution of GC-B dephosphorylation to achondroplasia (ACH) is unknown. GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    mice that express a glutamate-substituted version of GC-B that cannot be inactivated by dephosphorylation were bred with mice expressing FGFR3-G380R, the most common human ACH mutation, to determine if GC-B dephosphorylation is required for ACH. Crossing GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    mice with FGFR3\n                    <jats:sup>G380R\/G380R<\/jats:sup>\n                    mice increased naso-anal and long (tibia and femur), but not cranial, bone length twice as much as crossing GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    mice with FGFR3\n                    <jats:sup>WT\/WT<\/jats:sup>\n                    mice from 4 to 16 weeks of age. Consistent with increased GC-B activity rescuing ACH, long bones from the GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    \/FGFR3\n                    <jats:sup>G380R\/G380R<\/jats:sup>\n                    mice were not shorter than those from GC-B\n                    <jats:sup>WT\/WT<\/jats:sup>\n                    \/FGFR3\n                    <jats:sup>WT\/WT<\/jats:sup>\n                    mice. At 2 weeks of age, male but not female FGFR3\n                    <jats:sup>G380R\/G380R<\/jats:sup>\n                    mice had shorter long bones and smaller growth plate hypertrophic zones, whereas female but not male GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    mice had longer bones and larger hypertrophic zones. In 2-week-old males, crossing FGFR3\n                    <jats:sup>G380R\/G380R<\/jats:sup>\n                    mice with GC-B\n                    <jats:sup>7E\/7E<\/jats:sup>\n                    mice increased long bone length and hypertrophic zone area to levels observed in mice expressing WT versions of both receptors. We conclude that preventing GC-B dephosphorylation rescues reduced axial and appendicular skeleton growth in a mouse model of achondroplasia.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.147832","type":"journal-article","created":{"date-parts":[[2021,3,30]],"date-time":"2021-03-30T12:02:59Z","timestamp":1617105779000},"source":"Crossref","is-referenced-by-count":16,"title":["Prevention of guanylyl cyclase\u2013B dephosphorylation rescues achondroplastic dwarfism"],"prefix":"10.1172","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9162-3032","authenticated-orcid":true,"given":"Brandon M.","family":"Wagner","sequence":"first","affiliation":[{"name":"Departments of Integrative Biology and Physiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerid W.","family":"Robinson","sequence":"additional","affiliation":[{"name":"Biochemistry, Molecular Biology, and Biophysics, University of Minnesota, Minneapolis, Minnesota, 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