Article
Loss of Rai1 enhances hippocampal excitability and epileptogenesis in mouse models of Smith-Magenis syndrome.
Proceedings of the National Academy of Sciences of the United States of America - 25 Oct 2022
Chang Ya-Ting, Kowalczyk Max, Fogerson P Michelle, Lee Yu-Ju, Haque Minza, Adams Eliza L, Wang David C, DeNardo Laura A, Tessier-Lavigne Marc, Huguenard John R, Luo Liqun, Huang Wei-Hsiang
Abstract excerpt
Hyperexcitability of brain circuits is a common feature of autism spectrum disorders (ASDs). Genetic deletion of a chromatin-binding protein, retinoic acid induced 1 (RAI1), causes Smith-Magenis syndrome (SMS). SMS is a syndromic ASD associated with intellectual disability, autistic features, maladaptive behaviors, overt seizures, and abnormal electroencephalogram (EEG) patterns. The molecular and neural...
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