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CNS critical periods: implications for dystonia and other neurodevelopmental disorders
Jay Li, Sumin Kim, Samuel S. Pappas, William T. Dauer
Jay Li, Sumin Kim, Samuel S. Pappas, William T. Dauer
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CNS critical periods: implications for dystonia and other neurodevelopmental disorders

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Abstract

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.

Authors

Jay Li, Sumin Kim, Samuel S. Pappas, William T. Dauer

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Figure 2

TorsinB dictates developmental vulnerability to torsinA LOF–related NE blebbing.

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TorsinB dictates developmental vulnerability to torsinA LOF–related NE b...
Abnormal NE blebbing occurs during a developmental window when torsinB levels are low. Blebs resolve as torsinB levels rise during maturation. Simultaneous deletion of torsinA and torsinB prevents bleb resolution, highlighting how the torsinB expression level dictates susceptibility to torsinA LOF. Illustrated by Rachel Davidowitz.

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ISSN 2379-3708

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