4.7 Review

CNS critical periods: implications for dystonia and other neurodevelopmental disorders

Journal

JCI INSIGHT
Volume 6, Issue 4, Pages -

Publisher

AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/jci.insight.142483

Keywords

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Funding

  1. NIH [R01 NS109227, F31 NS113433, T32 GM007863, T32 GM007315]
  2. Tyler's Hope for a Dystonia Cure Foundation

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Critical periods in nervous system development are crucial stages where the system is sensitive to stimuli that aid in circuit maturation. The unique landscapes of the developing CNS create periods of susceptibility to pathogenic insults and responsiveness to therapy. A specific critical period has been identified in DYT1 dystonia, highlighting the harmful effects of the mutation and implications for future therapeutic development.
Critical periods are discrete developmental stages when the nervous system is especially sensitive to stimuli that facilitate c ircuit 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.

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