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    题名: Deficiency of cyclin-dependent kinase-like 5 causes spontaneous epileptic seizures in neonatal mice
    作者: 廖文霖
    Liao, Wenlin
    Kun-Ze Lee
    Jinchung Chen
    San-Hua Su
    Yuju Luo
    贡献者: 神科所
    日期: 2020-03
    上传时间: 2020-11-13 15:40:55 (UTC+8)
    摘要: Cyclin-dependent kinase-like 5 (CDKL5), an X-linked gene encoding a serine-threonine kinase, is enriched in the mammalian forebrain and critical for neuronal maturation and synaptic function. Mutations in this gene cause CDKL5 deficiency disorder (CDD), which is characterized by early-onset epileptic seizures, motor dysfunction and intellectual disability. Although several mouse models for CDD have been developed, overt spontaneous seizures have not been reported in adult mice with CDKL5 deficiency. Here, we present the first systematic study of spontaneous seizures in a mouse model of CDD. Through wireless electroencephalography (EEG) recording in a longitudinal manner, we observed epileptiform discharges in pups lacking CDKL5. The total number of spike events, the total burst number and burst duration were significantly increased in Cdkl5 null pups at the age of postnatal day 12 (P12). With age, these seizure-like discharges gradually diminished in mutant pups, while the discharges increased progressively in wild-type mice. Through simultaneous videotaping, Cdkl5 null pups exhibited seizure-like behaviors during prolonged bursts of discharges at P12, and showed increased ictal grasping and grip strength in adults. Notably, loss of CDKL5 remarkably down-regulated the phosphorylation of K+-Cl- co-transporter 2 (KCC2) in the cortex and hippocampus at P12. Our findings reveal a previously unidentified age-dependent phenotype of early-onset seizures in CDKL5-deficient neonatal mice, enhancing the translational value of CDD mouse model and providing a potential molecular target for early diagnosis and therapeutic development for CDD.
    關聯: bioRxiv, pp.1-37
    数据类型: article
    DOI 連結: https://doi.org/10.1101/2020.03.09.983981
    DOI: 10.1101/2020.03.09.983981
    显示于类别:[神經科學研究所] 期刊論文

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