Kato M, Hosokawa S, Tobimatsu S, Kuroiwa Y
J Cereb Blood Flow Metab. 1982 Dec;2(4):385-93. doi: 10.1038/jcbfm.1982.44.
Rolling mouse Nagoya (rolling), an experimental mutant mouse, is characterized by a marked incoordination of the hind limbs and disturbance of gait. These motor disturbances have been attributed to cerebellar dysfunction, and rolling, therefore, has been regarded as an animal model of hereditary cerebellar ataxia. However, definite evidence for this possibility has not yet been provided. In the present study, local cerebral glucose utilization (LCGU) was observed by means of the [14C]deoxyglucose method in rolling (rol/rol), as well as in behaviorally normal heterozygotes (+/rol) and normal controls (+/+), in order to study functional activity of the brain in these mice. A definite increase in LCGU was found in the globus pallidus, entopeduncular nucleus, subthalamic nucleus, and substantia nigra of rolling, bilaterally. A minimal decrease in LCGU was also found in the vermis of the cerebellum. These findings reflect the markedly hyperactive state of the basal ganglia and the minimally hypoactive state in the vermis of the cerebellum in rolling. It is concluded that the dysfunction in the basal ganglia is the major cause of the motor disturbances of rolling, and that rolling may be regarded as an animal model of extrapyramidal motor disturbance.
滚动小鼠名古屋品系（滚动小鼠）是一种实验性突变小鼠，其特征为后肢明显不协调和步态紊乱。这些运动障碍被认为是小脑功能障碍所致，因此，滚动小鼠被视为遗传性小脑共济失调的动物模型。然而，尚未有确凿证据支持这一可能性。在本研究中，为了研究这些小鼠大脑的功能活动，采用[14C]脱氧葡萄糖法观察了滚动小鼠（rol/rol）以及行为正常的杂合子（+/rol）和正常对照（+/+）的局部脑葡萄糖利用（LCGU）情况。结果发现，滚动小鼠双侧苍白球、内苍白球核、丘脑底核和黑质的LCGU明显增加。小脑蚓部的LCGU也有轻微下降。这些发现反映出滚动小鼠基底神经节明显活跃，而小脑蚓部轻微活动不足。研究得出结论，基底神经节功能障碍是滚动小鼠运动障碍的主要原因，滚动小鼠可被视为锥体外系运动障碍的动物模型。
