Craniosynostosis: genes and mechanisms.
نویسنده
چکیده
Enlargement of the skull vault occurs by appositional growth at the fibrous joints between the bones, termed cranial sutures. Relatively little is known about the developmental biology of this process, but genetically determined disorders of premature cranial suture fusion (craniosynostosis) provide one route to the identification of some of the key molecules involved. Mutations of the MSX2, FGFR1, FGFR2, FGFR3 and TWIST genes yield new insights, both into normal and abnormal cranial suture biogenesis and into problems of broad interest, such as the conservation of molecular pathways in development, and mechanisms of mutation and dominance.
منابع مشابه
Gene Expression Changes Between Patent and Fused Cranial Sutures in a Nonsyndromic Craniosynostosis Population
OBJECTIVE Craniosynostosis is a premature fusion of 1 or more cranial sutures. It may occur with additional morphological abnormalities (syndromic) or in isolation. Studies suggest that dysregulation of normal cell proliferation, differentiation, and migration has a role in isolated or nonsyndromic craniosynostosis but the molecular mechanisms remain unknown. The aim of this research is to iden...
متن کاملThe Effect of Inhalation Anesthesia with Hyperventilation and Total Intravenous Anesthesia on Intracranial Pressure Control in Pediatrics with Craniosynostosis Surgery: A Randomized Clinical Trial
Background: Intracranial pressure (ICP) control is one of the anesthesiologist challenges in craniosynostosis repair surgery, especially in multiple sutures involvement. The aim of this study was comparing the effect of two anesthesia methods including inhalation with hyperventilation and total intravenous anesthesia (TIVA) on ICP control and surgeon satisfaction in pediatrics with craniosynost...
متن کاملSpatio-temporal expression of FGFR 1, 2 and 3 genes during human embryo-fetal ossification
Mutations in FGFR 1-3 genes account for various human craniosynostosis syndromes, while dwarfism syndromes have been ascribed exclusively to FGFR 3 mutations. However, the exact role of FGFR 1-3 genes in human skeletal development is not understood. Here we describe the expression pattern of FGFR 1-3 genes during human embryonic and fetal endochondral and membranous ossification. In the limb bu...
متن کاملSignaling pathways in osteogenesis and osteoclastogenesis: Lessons from cranial sutures and applications to regenerative medicine
One of the simplest models for examining the interplay between bone formation and resorption is the junction between the cranial bones. Although only roughly a quarter of patients diagnosed with craniosynostosis have been linked to known genetic disturbances, the molecular mechanisms elucidated from these studies have provided basic knowledge of bone homeostasis. This work has translated to met...
متن کاملReliability of Visually Estimated Blood Loss with Hemoglobin Measurement: 200 Cases of Craniosynostosis Surgery
Background Bleeding is one of the most common complications of craniosynostosis surgery, which its appropriate management is associated with better post-operative outcomes. The aim of this retrospective study was to evaluate the visual estimated blood loss in intraoperative management of infants with craniosynostosis surgery. Materials and Methods This retrospective study was performed on 200 p...
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ورودعنوان ژورنال:
- Human molecular genetics
دوره 6 10 شماره
صفحات -
تاریخ انتشار 1997