Aikawa H, Nonaka I, Woo M, Tsugane T, Esaki K
Division of Ultrastructural Research, National Center of Neurology and Psychiatry, Tokyo, Japan.
Acta Neuropathol. 1988;76(4):366-72. doi: 10.1007/BF00686973.
Shaking rat Kawasaki (SRK), a newly discovered neurological mutant rat in the Wistar strain, is described. The abnormalities of SRK rats are transmitted as an autosomal recessive trait. The neurological signs are shaking of the body and an ataxic-paretic gait from day 10 postnatal. The affected rats survive for about 1 month. Macroscopically, the cerebellum is small and frequently the vermis and paraflocculus lacking. The most conspicuous histological finding in the central nervous system is malposition of the neurons in the cerebral cortex, hippocampus and cerebellum. Myelination and synapse formation are intact. Abnormal myelinated fibers are present in the molecular layer of the cerebral cortex and in the central gray matter of the spinal cord. These morphological abnormalities resemble those reported in the reeler mutant mouse. SRK rats are another good animal model of human congenital malformations with neuronal migration disorders.
描述了摇尾大鼠川崎病(SRK),一种新发现的Wistar品系神经突变大鼠。SRK大鼠的异常作为常染色体隐性性状遗传。神经症状从出生后第10天开始表现为身体颤抖和共济失调性轻瘫步态。患病大鼠存活约1个月。宏观上,小脑较小,蚓部和旁绒球常缺失。中枢神经系统最明显的组织学发现是大脑皮层、海马体和小脑中神经元位置异常。髓鞘形成和突触形成正常。大脑皮层分子层和脊髓中央灰质中存在异常髓鞘纤维。这些形态学异常与reeler突变小鼠中报道的相似。SRK大鼠是人类先天性神经元迁移障碍畸形的另一种良好动物模型。