Wilkie A O
Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK.
Indian J Pediatr. 1996 May-Jun;63(3):351-6. doi: 10.1007/BF02751527.
The post eighteen months have been exciting time for craniosynostosis research. In a rapid flurry of publications, mutations of fibroblast growth factor receptors (FGFRs) have been identified in three of the best known craniosynostosis syndromes, namely Apert, Crouzon and Pfeiffer syndromes, as well as in Jackson-Weiss syndrome and thanatophoric dysplasia. These findings open many new avenues for craniosynostosis research including studies of diagnosis, pathogenesis, and mutagenesis. Here the major findings and their implications have been briefly reviewed.
在过去的十八个月里,颅缝早闭症的研究进入了令人兴奋的阶段。在一系列迅速发表的论文中,成纤维细胞生长因子受体(FGFRs)的突变已在三种最著名的颅缝早闭症综合征中被发现,即Apert综合征、Crouzon综合征和Pfeiffer综合征,以及Jackson-Weiss综合征和致死性骨发育不全。这些发现为颅缝早闭症的研究开辟了许多新途径,包括诊断、发病机制和诱变研究。在此,主要研究结果及其意义已被简要综述。