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We observed that developmental abnormalities of the cranial base synchondroses involving proliferative chondrocytes are important in craniofacial growth and development. Neonatal restitution of \u03b2-hexosaminidase in mutant mice by gene therapy successfully ameliorated the attendant skeletal defects and restored craniofacial morphology in vivo, suggesting this as a critical temporal window in craniofacial development. Analysis of our data implicates parathyroid-related peptide (PTHrP) and cyclo-oxygenase-2 (COX-2) as possible factors underlying the development of the aforementioned skeletal defects. Hence, timely restitution of a genetic deficiency or, alternatively, the restoration of PTHrP or cyclo-oxygenase activity by the administration of PTH and\/or non-steroidal anti-inflammatory drugs or COX-2 selective inhibitors to affected individuals may prove beneficial in the management of midface retrusion.<\/jats:p>","DOI":"10.1177\/154405910708601008","type":"journal-article","created":{"date-parts":[[2009,1,8]],"date-time":"2009-01-08T19:39:42Z","timestamp":1231443582000},"page":"956-961","source":"Crossref","is-referenced-by-count":6,"title":["The Cranial Base in Craniofacial Development: a Gene Therapy Study"],"prefix":"10.1177","volume":"86","author":[{"given":"S.","family":"Kyrkanides","sequence":"first","affiliation":[{"name":"Departments of Dentistry,"},{"name":"Neurobiology & Anatomy, and"},{"name":"Department of Orthopedics and Center for Musculo-skeletal Research, School of Medicine and Dentistry, University of Rochester Medical Center, 625 Elmwood Ave., Rochester NY 14620, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Kambylafkas","sequence":"additional","affiliation":[{"name":"Departments of Dentistry,"},{"name":"Neurobiology & Anatomy, and"},{"name":"Department of Orthopedics and Center for Musculo-skeletal Research, School of Medicine and Dentistry, University of Rochester Medical Center, 625 Elmwood Ave., Rochester NY 14620, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.H.","family":"Miller","sequence":"additional","affiliation":[{"name":"Departments of Dentistry,"},{"name":"Neurobiology & Anatomy, and"},{"name":"Department of Orthopedics and Center for Musculo-skeletal Research, School of Medicine and Dentistry, University of Rochester Medical Center, 625 Elmwood Ave., Rochester NY 14620, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.H.","family":"Tallents","sequence":"additional","affiliation":[{"name":"Departments of Dentistry,"},{"name":"Neurobiology & Anatomy, and"},{"name":"Department of Orthopedics and Center for Musculo-skeletal Research, School of Medicine and Dentistry, University of Rochester Medical Center, 625 Elmwood Ave., Rochester NY 14620, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.E.","family":"Puzas","sequence":"additional","affiliation":[{"name":"Departments of Dentistry,"},{"name":"Neurobiology & Anatomy, and"},{"name":"Department of Orthopedics and Center for Musculo-skeletal Research, School of Medicine and Dentistry, University of Rochester Medical Center, 625 Elmwood Ave., Rochester NY 14620, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2007,10,1]]},"reference":[{"key":"BAUME-1970","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9416(70)90143-0"},{"key":"DE-CROMBRUGGHE-ETAL-2000","doi-asserted-by":"publisher","DOI":"10.1016\/S0945-053X(00)00094-9"},{"key":"FUJITA-ETAL-2004","doi-asserted-by":"publisher","DOI":"10.1177\/154405910408300313"},{"key":"FURUTA-AND-JEE-1986","doi-asserted-by":"publisher","DOI":"10.1002\/ar.1092150313"},{"key":"GAKUNGA-ETAL-2000","unstructured":"Gakunga PT, Kuboki Y, Opperman LA -2000- Hyaluronan is essential for the expansion of the cranial base growth plates. 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