Krémarik P, Strazielle C, Lalonde R
Université de Nancy 1, Laboratoire de Biologie Cellulaire du Développement, Vandoeuvre-les-Nancy, France.
Eur J Neurosci. 1998 Sep;10(9):2802-9. doi: 10.1111/j.1460-9568.1998.00290.x.
Hot-foot mutant mice are characterized by defective arborization of Purkinje cell dendrites, resulting in ataxia of gait and deficits of equilibrium. Regional brain variations of cytochrome oxidase (CO) activity were analysed for the purpose of identifying those brain regions with abnormal metabolic activity as a secondary consequence to the cerebellar alteration. In addition, the possible relation between CO activity and motor deficits was evaluated. By comparison to normal littermate controls of the same background strain, hot-foot mutants had higher CO activity in the molecular layer of the cerebellum, the ventrolateral and midline thalamic nuclei, as well as in the frontal eye field. There was no other alteration of CO activity in the hot-foot brain. No linear correlation was discerned between CO activity in the molecular layer of the cerebellum and the ventrolateral and midline nuclei on one hand, and motor coordination performance on the other. These results indicate regionally selective abnormalities of metabolic activity in a cerebellar mutant with defective dendritic arborization of the Purkinje cell.
热足突变小鼠的特征是浦肯野细胞树突分支缺陷,导致步态共济失调和平衡能力受损。为了确定作为小脑改变的继发后果而具有异常代谢活动的脑区,对细胞色素氧化酶(CO)活性的区域脑差异进行了分析。此外,还评估了CO活性与运动缺陷之间的可能关系。与相同背景品系的正常同窝对照相比,热足突变体在小脑分子层、腹外侧和中线丘脑核以及额叶眼区具有较高的CO活性。热足小鼠的脑中CO活性没有其他改变。一方面,小脑分子层以及腹外侧和中线核中的CO活性与另一方面的运动协调性能之间没有发现线性相关性。这些结果表明,在一个具有浦肯野细胞树突分支缺陷的小脑突变体中,代谢活动存在区域选择性异常。