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Alteration of neuronal network in the central nervous system of a dystonic mutant mouse, "Wriggle mouse Sagami"

肌张力障碍突变小鼠“Wriggle mouse Sagami”中枢神经系统神经元网络的改变

基本信息

批准号：
02670023
负责人：
INOUE Yoshiro
金额：
$ 1.41万
依托单位：
Hokkaido University
依托单位国家：
日本
项目类别：
Grant-in-Aid for General Scientific Research (C)
财政年份：
1990
资助国家：
日本
起止时间：
1990 至 1991
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-02670023/
关键词：
Wriggle mouse sagami Congenital disease model Mutant Dystonic mouse Plasticity Cerebellum Biocytin DiI 遺伝性疾集患モデル

项目摘要

"Wriggle Mouse Sagami(WMS)" is a new autosomal recessive mutant characteristic in remarkable dystonic movements, which initially appear 10 days after birth and progress until 12 weeks of age. In spite of the severe motor disorders. the general cytoarchitecture of the CNS and fiber connections of the motor system had been considered to be normal. In this project we made clear the alteration of the synapse architecture in the CNS by immunohistochemistry for IP_3 receptor protein, GAD(glutamic acid decarboxylase), GABA and serotonin and electron microscopy. The results are as follows : (I)The cytoarchitecture and fiber connections in the CNS appeared normal according to the HRP- or biocytin-labelling method ; (2)parallel fibers of the WMS cerebellum were apparently reduced and the synaptic terminals on tlie dendritic spines of Purkinje cells decreased in number. even at 17 days after birth : (2)GAD'or GABA-immunopositive synaptic boutons or bouton-like structures, on the other hand, remarkably increased in the molecular, , -Purkinje cell, and granule cell layers of the WMS cerebellum : (3)the synapse architecture in the motor cortex and the molecular layer of the hippocampus CAl was clearly different from that of the control mouse ; (4)serotonergic neurons in the raphe nuclei appeared almost the same distribution as those of the control ones.Consequently, the WMS is considered to be a new mutation with abnormal synaptic formation in spite of the normal cytoarchitecture of the neurons and fiber connection, and could be a model animal of the dystonia musculorm deformans of the human beings. The WMS is also possibly useful for investigating tlie mechanism of synaptic formation. information conducting system at synaptic sites, or environmental factors maintaining synaptic function in the neuronal circuits of the CNS.

“Wriggle Mouse Sagami（WMS）”是一种新的常染色体隐性突变，以显著的肌张力障碍运动为特征，最初出现在出生后10天，并发展到12周龄。尽管有严重的运动障碍。中枢神经系统的总体细胞结构和运动系统的纤维连接被认为是正常的。本课题通过免疫组化、电镜和谷氨酸脱羧酶（GAD）、氨基丁酸（GABA）、5 -羟色胺（5 -羟色胺）等免疫组化方法，明确了中枢神经系统突触结构的改变。结果表明：(1)经HRP或生物细胞素标记法观察，大鼠中枢神经系统的细胞结构和纤维连接正常；(2) WMS小脑平行纤维明显减少，浦肯野细胞树突棘突触末端数量减少。即使在出生后17天：(2)另一方面，在WMS小脑的分子层、-浦肯野细胞层和颗粒细胞层中，GAD'或gaba免疫阳性的突触钮扣或钮扣样结构显著增加；(3)运动皮质和海马CAl分子层的突触结构与对照小鼠明显不同；(4)中缝核5 -羟色胺能神经元的分布与对照组基本相同。因此，WMS被认为是一种新的突变，尽管神经元的细胞结构和纤维连接正常，但突触形成异常，可能是人类肌张力障碍畸形的模型动物。WMS也可能用于研究突触形成的机制。突触部位的信息传导系统，或中枢神经系统神经元回路中维持突触功能的环境因素。