Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice
Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice
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DOI：
10.1538/expanim.56.161
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发表时间：
2007-04-01
影响因子：
2.4
通讯作者：
Serikawa, Tadao
中科院分区：
文献类型：
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作者：
Gohma, Hiroshi;Kuramoto, Takashi;Serikawa, Tadao
自发性癫痫大鼠（SER）是震颤和zitter突变的双突变体，表现出自发发生的失神样和强直性癫痫发作。多项证据表明，Aspa和Atrn突变的联合效应是导致SER癫痫表型的最可能原因。为了解决这个问题，我们生产了一种新的双突变小鼠系，携带纯合Aspa基因敲除和Atrn（mg-3 J）突变等位基因。Aspa/Atrn双突变小鼠表现出失神样和强直性癫痫发作，其特征在于EEG上出现5-7 Hz棘波样复合物和低电压快波。这些结果直接表明，Aspa和Atrn基因功能的同时丧失导致小鼠癫痫发作，并表明Aspa和Atrn缺陷可能是SER中癫痫发作的原因。
The Spontaneously Epileptic Rat (SER), a double-mutant for tremor and zitter mutations, shows spontaneous occurrences of absence-like and tonic seizures. Several lines of evidence suggest that the combined effect of Aspa and Atrn mutations is the most likely cause of the epileptic phenotype of the SER. To address this issue, we produced a new double-mutant mouse line carrying both homozygous Aspa-knockout and Atrn(mg-3J) mutant alleles. The Aspa/Atrn double-mutant mice exhibited absence-like and tonic seizures that were characterized by the appearance of 5-7 Hz spike-wave-like complexes and low voltage fast waves on EEGs. These results demonstrate directly that the simultaneous loss of the Aspa and Atrn gene functions causes epileptic seizures in the mouse and suggest that both Aspa and Atrn deficiencies might be responsible for epileptic seizures in the SER.