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However, the exact function of ADAMTSL2 is unknown. To elucidate the role of this protein in skeletal development, we generated complementary knockout (KO) mouse models with either total or chondrocyte Adamtsl2 deficiency. We observed that the Adamtsl2 KO mice displayed skeletal abnormalities reminiscent of the human phenotype. Adamtsl2 deletion affected the growth plate formation with abnormal differentiation and proliferation of chondrocytes. In addition, a TGF\u2010\u03b2 signaling impairment in limbs lacking Adamtsl2 was demonstrated. Further investigations revealed that Adamtsl2 KO chondrocytes failed to establish a microfibrillar network composed by fibrillin1 and latent TGF\u2010\u03b2 binding protein 1 fibrils. Chondrocyte Adamtsl2 KO mice also exhibited dwarfism. These studies uncover the function of Adamtsl2 in the maintenance of the growth plate ECM by modulating the microfibrillar network.\u2014Delhon, L., Mahaut, C., Goudin, N., Gaudas, E., Piquand, K., Le Goff, W., Cormier\u2010Daire, V., Le Goff, C. Impairment of chondrogenesis and microfibrillar network in Adamtsl2 deficiency. FASEB J. 33, 2707\u20132718 (2019).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.201800753rr","type":"journal-article","created":{"date-parts":[[2018,10,10]],"date-time":"2018-10-10T14:13:12Z","timestamp":1539180792000},"page":"2707-2718","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Impairment of chondrogenesis and microfibrillar network in Adamtsl2 deficiency"],"prefix":"10.1096","volume":"33","author":[{"given":"Laure","family":"Delhon","sequence":"first","affiliation":[{"name":"INSERM Unit\u00e9 Mixte de Recherche (UMR) 1163 Laboratory of Molecular and Physiopathological Bases of Osteochondrodysplasia Imagine Institute Paris Descartes University-Sorbonne Paris Cit\u00e9 Paris 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