Hyperspiny Purkinje cell, a new neurological mutation in the mouse.
Hyperspiny Purkinje cell, a new neurological mutation in the mouse.
复制标题
超刺浦肯野细胞，小鼠中的一种新的神经突变。
作者：
J. Guénet;C. Sotelo;J. Mariani
浦肯野细胞（Hyperspiny Purkinje cell, hpc）是一种新的常染色体隐性突变小鼠。纯合子在出生后10天左右表现出异常的运动行为，以小脑症状为主。形态学分析显示小脑略有减少，浦肯野细胞选择性改变。所有这些神经元都有一个萎缩的树突树。近端树突和细胞体不是光滑的，而是布满了刺。属于该神经元群的大多数轴突终末发生进行性退行性变化。只有一小部分浦肯野细胞死亡。电生理研究显示攀爬纤维与浦肯野细胞之间的突触功能正常，未见明显异常。
Hyperspiny Purkinje cell (hpc) is a new autosomal recessive mutation of the laboratory mouse. Homozygotes exhibit abnormal motor behavior, with predominance of cerebellar symptoms, about 10 days after birth. Morphological analysis disclosed a slightly reduced cerebellum with selective alteration of Purkinje cells. All these neurons have an atrophic dendritic tree. The proximal dendritic branches and the cell bodies, instead of being smooth, are studded with spines. Most of the axon terminals belonging to this neuronal population undergo progressive degenerative changes. Cell death is observed in only a small proportion of Purkinje cells. Electrophysiological study revealed that the synapses between climbing fibers and Purkinje cells are functional with no gross abnormality.