Analysis of seizures in a new epileptic model rat strain

新型癫痫模型大鼠品系的癫痫发作分析

基本信息

批准号：
10680780
负责人：
TSUJI Shigekatsu
金额：
$ 2.24万
依托单位：
Wakayama Medical University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
1998
资助国家：
日本
起止时间：
1998 至 1999
项目状态：
已结题

项目摘要

We found a new epileptic mutant rat and established a new epileptic rat strain, WER (Wakayama Epileptic Rat). In order to evaluate this strain as an animal model for human epilepsy, we analyzed type of seizures and electroencephalograms (EEG) at paroxysms. The WER rats spontaneously exhibited both tonic-clonic convulsion and absence-like seizures. The spike-and-wave discharges were characterized to 5.1±0.4Hz, average with standard error, in the absence-like seizures. Low-voltage fast waves were recorded in the tonic-clinic convulsions. These EEG patterns were resembled to those of human epilepsies. The onset of this disorder was estimated between 25 to 70 days of age. Some WER rats died suddenly between the onset period. Therefore sudden death at the seizure occurrence was assumed. After the sensitive period, the unexpected death was not observed and stable seizure occurrence was observed in the WER strain. Genetic analyses of the disorder siggested that the seizure occurrence was controlled by an autosomal single recessive gene. The penetrance of the gene was estimated to 86%. The SER and TM rats, and swe mice were reported that exhibited two types of seizures spontaneously by a single gene defect. The seizure phenotype of WER was resembled to those of the SER and TM rats, but other characteristics including morphological characters were not identical to those models. Therefore a new epileptic gene was suggested in the WER rats. The WER strain was suggested to be a useful animal model for inherited human epilepsy.

我们发现了一种新的癫痫突变大鼠，并建立了一种新的癫痫大鼠菌株（Wakayama癫痫大鼠）。为了评估这种菌株作为人癫痫的动物模型，我们分析了阵发性的癫痫发作和脑电图（EEG）的类型。 wer大鼠自发暴露了强直性惊厥和缺失样癫痫发作。在缺席的癫痫发作的情况下，尖峰和波排放的表征为5.1±0.4Hz，平均误差为平均值。低压快速波记录在补品 - 临床抽搐中。这些脑电图与人类癫痫的模式相似。该疾病的发作估计在25至70天之间。一些大鼠在开始时期突然死亡。因此，假定发生癫痫发作时的猝死。在敏感的时期之后，未观察到意外死亡，并且在WER菌株中观察到稳定的癫痫发作。该疾病的遗传分析表明，癫痫发作的发生是由常染色体单一隐性基因控制的。该基因的渗透率估计为86％。据报道，SER和TM大鼠以及SWEE小鼠暴露了两种由单个基因缺陷赞助的癫痫发作。 WER的癫痫发作表型与SER和TM大鼠的癫痫发作相似，但是包括形态特征在内的其他特征与这些模型并不相同。因此，在大鼠中提出了一种新的癫痫基因。认为菌株是遗传性癫痫的有用动物模型。