Medical Scientist Training Program, University of Michigan, Ann Arbor, Michigan, USA.
Cellular and Molecular Biology Graduate Program, University of Michigan, Ann Arbor, Michigan, USA.
JCI Insight. 2021 Feb 22;6(4):142483. doi: 10.1172/jci.insight.142483.
Critical periods are discrete developmental stages when the nervous system is especially sensitive to stimuli that facilitate circuit maturation. The distinctive landscapes assumed by the developing CNS create analogous periods of susceptibility to pathogenic insults and responsiveness to therapy. Here, we review critical periods in nervous system development and disease, with an emphasis on the neurodevelopmental disorder DYT1 dystonia. We highlight clinical and laboratory observations supporting the existence of a critical period during which the DYT1 mutation is uniquely harmful, and the implications for future therapeutic development.
关键期是神经系统对外界刺激特别敏感的离散发育阶段,这些刺激有助于促进回路成熟。发育中的中枢神经系统所呈现的独特景观,创造了类似的易受致病因素侵袭和对治疗有反应的敏感时期。在这里,我们回顾了神经系统发育和疾病中的关键期,重点介绍了神经发育障碍 DYT1 型肌张力障碍。我们强调了支持在 DYT1 突变具有独特危害性的关键期存在的临床和实验室观察结果,以及对未来治疗开发的影响。