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首页> 外文学位 >Pathogenic fibroblast growth factor receptor 2 signaling adversely affects diverse cellular processes during embryonic and post-natal development of the mouse cerebral cortex.
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Pathogenic fibroblast growth factor receptor 2 signaling adversely affects diverse cellular processes during embryonic and post-natal development of the mouse cerebral cortex.

机译：致病性成纤维细胞生长因子受体2信号在小鼠大脑皮层的胚胎和出生后发育过程中对多种细胞过程产生不利影响。

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摘要

Activating mutations in fibroblast growth factor receptors (FGFR) -1, -2, -3 cause craniosynostosis, the premature fusion of one or more cranial sutures, in Apert, Crouzon, Pfeiffer, Muenke, and Beare-Stevenson cutis gyrata syndromes. These conditions are also characterized by other skeletal anomalies and variable effects on brain development and function. The variability of central nervous system defects and the occurrence of severe cognitive impairment despite early surgical intervention, particularly in Apert syndrome, led us to hypothesize that the activating FGFR mutations have a direct, adverse effect on brain development. In this study we looked for aberrations in diverse cellular processes affected by Fgf signaling in the developing telencephalon of an Fgfr2+/S252W Apert mouse model to uncover the mechanisms by which these defects occur. We present evidence that in the Apert mouse model, the radial glial cells to intermediate progenitor transition is depressed during mid-to-late neurogenesis. We also observed a significant increase in apoptosis in the anterior forebrain in Apert newborns. Lastly, an increase in the migration of astrocytes to the dorsolateral cortex by post-natal day five was observed. These results suggest that important physiological roles of Fgfr2 signaling in the development of the cerebral cortex are adversely affected by syndromic activating mutations of Fgfr2, which may lead to gross brain abnormalities.
机译：成纤维细胞生长因子受体（FGFR）-1，-2，-3的激活突变会导致颅骨突增生症，即一种或多种颅骨缝线在Apert，Crouzon，Pfeiffer，Muenke和Beare-Stevenson cutis gyrata综合征中的过早融合。这些疾病的特征还在于其他骨骼异常以及对大脑发育和功能的各种影响。尽管进行了早期外科手术干预，特别是在Apert综合征中，中枢神经系统缺陷的变化和严重的认知障碍的发生，使我们假设激活的FGFR突变对大脑发育有直接的不利影响。在这项研究中，我们在Fgfr2 + / S252W Apert小鼠模型的正在发展的端脑中寻找受Fgf信号影响的各种细胞过程中的畸变，以揭示这些缺陷发生的机制。我们目前的证据表明，在Apert小鼠模型中，放射状神经胶质细胞向中间祖细胞的过渡在中晚期神经形成过程中被抑制。我们还观察到Apert新生儿的前脑细胞凋亡显着增加。最后，观察到出生后第五天星形胶质细胞向背外侧皮层的迁移增加。这些结果表明，Fgfr2信号激活的突变对Fgfr2信号在大脑皮层发育中的重要生理作用产生不利影响，这可能导致严重的脑部异常。

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  • 作者

    Heitman, Nicholas.;

  • 作者单位

    Icahn School of Medicine at Mount Sinai.;

  • 授予单位 Icahn School of Medicine at Mount Sinai.;
  • 学科 Biology Genetics.;Biology Neuroscience.;Health Sciences Human Development.
  • 学位 M.S.
  • 年度 2013
  • 页码 36 p.
  • 总页数 36
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类
  • 关键词

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