Tyrosine kinase receptor c-ros-oncogene 1 mediates Twist-1 haploinsufficiency induced craniosynostosis in children: A novel therapeutic target

酪氨酸激酶受体c-ros-癌基因1介导Twist-1单倍体不足引起的儿童颅缝早闭：一个新的治疗靶点

• 批准号: nhmrc : 1120989
• 负责人: Prof Andrew Zannettino
• 金额: $ 38.41万
• 依托单位: The University of Adelaide
• 依托单位国家: 澳大利亚
• 项目类别: Project Grants
• 财政年份: 2017
• 资助国家: 澳大利亚
• 起止时间: 2017-01-01 至 2019-12-31
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/tyrosine-kinase-receptor-therapeutic-target/946660
• 关键词: Tyrosine; kinase; receptor; ros; oncogene

Children with Saethre-Chotzen syndrome exhibit premature fussed coronal sutures, and other skull/ skeletal malformations. Surgical intervention is the only treatment option to ensure optimal cognitive and skeletal development. Our studies have identified a candidate molecular pathway that regulates bone formation by cranial bone cells from these patients. Targeting these key molecular signalling components with chemical inhibitors will help prevent the premature fusion of cranial sutures.

患有 Saethre-Chotzen 综合征的儿童表现出冠状缝过早脱落和其他颅骨/骨骼畸形。手术干预是确保最佳认知和骨骼发育的唯一治疗选择。我们的研究已经确定了一种候选分子途径，可以调节这些患者的颅骨细胞的骨形成。用化学抑制剂靶向这些关键分子信号成分将有助于防止颅缝过早融合。