{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T15:05:22Z","timestamp":1781103922864,"version":"3.54.1"},"reference-count":0,"publisher":"National Academy of Sciences","issue":"18","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1990,9]]},"abstract":"<jats:p>Osteogenesis imperfecta type I is a mild, dominantly inherited, connective tissue disorder characterized by bone fragility. Mutations in type I collagen account for all known cases. In Mov-13 mice, integration of a murine retrovirus within the first intron of the alpha 1(I) collagen gene results in a null allele blocked at the level of transcription. This study demonstrates that mutant mice heterozygous for the null allele are a model of osteogenesis imperfecta type I. A defect in type I collagen production is associated with dominant-acting morphological and functional defects in mineralized and nonmineralized connective tissue and with progressive hearing loss. The model provides an opportunity to investigate the effect of a reduced amount of type I collagen on the structure and integrity of extracellular matrix. It also may represent a system in which therapeutic strategies to strengthen connective tissue can be developed.<\/jats:p>","DOI":"10.1073\/pnas.87.18.7145","type":"journal-article","created":{"date-parts":[[2006,5,31]],"date-time":"2006-05-31T07:26:26Z","timestamp":1149060386000},"page":"7145-7149","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":125,"title":["Transgenic mouse model of the mild dominant form of osteogenesis imperfecta."],"prefix":"10.1073","volume":"87","author":[{"given":"J","family":"Bonadio","sequence":"first","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T L","family":"Saunders","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Tsai","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S A","family":"Goldstein","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Morris-Wiman","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L","family":"Brinkley","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D F","family":"Dolan","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R A","family":"Altschuler","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"J E","family":"Hawkins","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J F","family":"Bateman","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Department of Pathology, University of Michigan, Ann Arbor 48109-0650."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1990,9]]},"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.87.18.7145","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T13:23:31Z","timestamp":1649856211000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.87.18.7145"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1990,9]]},"references-count":0,"journal-issue":{"issue":"18","published-print":{"date-parts":[[1990,9]]}},"alternative-id":["10.1073\/pnas.87.18.7145"],"URL":"https:\/\/doi.org\/10.1073\/pnas.87.18.7145","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1990,9]]},"assertion":[{"value":"1990-09-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}