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It is still unknown if the collagen defect also affects lung development and structure, causing lung hypoplasia in OI type II. The aim of this study was to investigate the intrinsic characteristics of OI embryonic lung parenchyma and to determine whether altered collagen type I may compromise airway development and lung structure. Lung tissue from nine fetuses with OI type II and six control fetuses, matched by gestational age, was analyzed for TTF\u20101 and collagen type I expression by immunohistochemistry, to evaluate the state of lung development and amount of collagen. The differentiation of epithelium into type 2 pneumocytes during embryonic development was premature in OI type II fetuses compared to controls (p\u2009&lt;\u20090.05). Collagen type I showed no significant differences between the two groups. However, the amount of alpha2(I) chains was higher in fetuses with OI and the ratio of alpha1(I) to alpha2(I) lower in OI compared to controls. Cell differentiation during lung embryonic development in patients with OI type II is premature and impaired. This may be the underlying cause of pulmonary hypoplasia. Altered cell differentiation can be secondary to mechanical chest factors or a consequence of disrupted type I collagen synthesis. Our findings suggest that collagen type I is a biochemical regulator of pulmonary cell differentiation, influencing lung development.<\/jats:p>","DOI":"10.14814\/phy2.15737","type":"journal-article","created":{"date-parts":[[2023,7,4]],"date-time":"2023-07-04T07:04:54Z","timestamp":1688454294000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Altered collagen I and premature pulmonary embryonic differentiation in patients with<scp>OI<\/scp>type<scp>II<\/scp>"],"prefix":"10.14814","volume":"11","author":[{"given":"S.","family":"Storoni","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Section Endocrinology Amsterdam UMC Location Vrije Universiteit Amsterdam Amsterdam The Netherlands"},{"name":"Amsterdam Movement Sciences Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Celli","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Section Endocrinology Amsterdam UMC Location Vrije Universiteit Amsterdam Amsterdam The Netherlands"},{"name":"Amsterdam Movement Sciences Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Breur","sequence":"additional","affiliation":[{"name":"Department of Pathology Amsterdam University Medical Centre Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Micha","sequence":"additional","affiliation":[{"name":"Amsterdam Movement Sciences Amsterdam The Netherlands"},{"name":"Department of Human Genetics Amsterdam UMC Location Vrije Universiteit Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. 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