Gli3 mice exhibit lambdoid suture craniosynostosis which results from altered osteoprogenitor proliferation and differentiation
نویسندگان
چکیده
Department of Orthodontics, Institute of Dentistry, 00014 University of Helsinki, PO Box 41 (Mannerheimintie 172), Finland, Department of Craniofacial Development and Department of Orthodontics, King’s College, Floor 28 Guy’s Tower, London SE1 9RT, UK, Oral and Maxillofacial Diseases, Helsinki University Central Hospital, Helsinki, Finland, Developmental Biology Program, Childrens Hospital Los Angeles/The Saban Research Institute, Los Angeles, CA 90027, USA, A∗STAR Institute of Molecular and Cell Biology, 61 Biopolis Drive, Singapore 138673, Singapore and Excellence Cluster in Cardio-Pulmonary System, University Justus Liebig, Giessen, Germany
منابع مشابه
Loss-of-Function of Gli3 in Mice Causes Abnormal Frontal Bone Morphology and Premature Synostosis of the Interfrontal Suture
Greig cephalopolysyndactyly syndrome (GCPS) is an autosomal dominant disorder with polydactyly and syndactyly of the limbs and a broad spectrum of craniofacial abnormalities. Craniosynostosis of the metopic suture (interfrontal suture in mice) is an important but rare feature associated with GCPS. GCPS is caused by mutations in the transcription factor GLI3, which regulates Hedgehog signaling. ...
متن کاملRegulation of Calvarial Osteogenesis by Concomitant De-repression of GLI3 and Activation of IHH Targets
Loss-of-function mutations in GLI3 and IHH cause craniosynostosis and reduced osteogenesis, respectively. In this study, we show that Ihh ligand, the receptor Ptch1 and Gli transcription factors are differentially expressed in embryonic mouse calvaria osteogenic condensations. We show that in both Ihh-/- and Gli3Xt-J/Xt-J embryonic mice, the normal gene expression architecture is lost and this ...
متن کاملSurgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients.
OBJECTIVE Surgery for craniosynostosis has evolved rapidly over the past two decades, with increased emphasis on early, extensive operations. Older published series may not accurately reflect more recent experience. Our study was designed to analyze outcome in a large series of consecutive patients treated recently at a single center. METHODS We reviewed 250 consecutive patients who underwent...
متن کاملCraniosynostosis.
Skull deformity in infants continues to be a diagnostic and therapeutic challenge. Deformational plagiocephaly is a common and somewhat benign cause of skull deformity in infants that must be distinguished from the more serious craniosynostosis, which occurs alone or as a syndrome. Examining an infant's head from above can help the physician distinguish true lambdoid synostosis from deformation...
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BACKGROUND Radiography, typically the first-line imaging study for diagnosis of craniosynostosis, exposes infants to ionizing radiation. We aimed to compare the accuracy of cranial ultrasound (CUS) with radiography for the diagnosis or exclusion of craniosynostosis. METHODS Children aged 0 to 12 months who were assessed for craniosynostosis during 2011-2013 by using 4-view skull radiography a...
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