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通过微管相关蛋白驱动蛋白轻链3的表达揭示正常小鼠和扰乱小鼠的小脑核解剖结构。

The anatomy of the cerebellar nuclei in the normal and scrambler mouse as revealed by the expression of the microtubule-associated protein kinesin light chain 3.

作者信息

Chung Seunghyuk, Zhang Ying, Van Der Hoorn Frans, Hawkes Richard

机构信息

Department of Cell Biology and Anatomy, Hotchkiss Brain Institute, and Genes and Development Research Group, Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.

出版信息

Brain Res. 2007 Apr 6;1140:120-31. doi: 10.1016/j.brainres.2006.01.100.

DOI:10.1016/j.brainres.2006.01.100
PMID:17447264
Abstract

Conventional kinesin is a motor protein complex including two heavy chains and two light chains (KLC). Junco et al. (Junco, A., Bhullar, B., Tarnasky, H.A. and van der Hoorn, F.A., 2001. Kinesin light-chain KLC3 expression in testis is restricted to spermatids. Biol. Reprod. 64, 1320-1330). recently reported the isolation of a novel KLC gene, klc3. In the present report, immunohistochemistry has been used to characterize the expression of KLC3 in the cerebella of normal and scrambler (scm) mutant mice. In cryostat sections through the cerebellum of the normal adult mouse immunoperoxidase stained for KLC3, reaction product is deposited in the nuclei and somata of deep cerebellar nuclear neurons. No other structures are stained in the cerebellum. Strong and specific KLC3 expression is observed in the adult cerebellum in all three major cerebellar nuclei--medial, interposed, and lateral. Double immunofluorescence studies reveal that KLC3 immunoreactivity is colocalized with both endosomes and GW bodies. KLC3 immunohistochemistry has been exploited to study the organization of the cerebellar nuclei in scrambler mice, in which disruption of the mdab1 gene results in severe foliation defects due to Purkinje cell ectopia, with most Purkinje cells clumped in centrally located clusters. Despite the severe failure of Purkinje cell migration, the cerebellar nuclei appear normal in scrambler mutant mice, suggesting that their topography is dependent neither on normal Purkinje cell positioning nor the Reelin signaling pathway.

摘要

传统的驱动蛋白是一种马达蛋白复合体,包含两条重链和两条轻链(KLC)。Junco等人(Junco, A., Bhullar, B., Tarnasky, H.A. 和van der Hoorn, F.A., 2001. 驱动蛋白轻链KLC3在睾丸中的表达仅限于精子细胞。《生物繁殖》64, 1320 - 1330)最近报道了一个新的KLC基因klc3的分离。在本报告中,免疫组织化学被用于表征KLC3在正常和扰乱(scm)突变小鼠小脑的表达。在通过正常成年小鼠小脑的冰冻切片中,用免疫过氧化物酶对KLC3进行染色,反应产物沉积在小脑深部核神经元的细胞核和胞体中。小脑中没有其他结构被染色。在成年小脑中,在所有三个主要的小脑核——内侧核、中间核和外侧核中都观察到了强烈且特异的KLC3表达。双重免疫荧光研究表明,KLC3免疫反应性与内体和GW小体都共定位。KLC3免疫组织化学已被用于研究扰乱小鼠中小脑核的组织结构,在这种小鼠中,mdab1基因的破坏由于浦肯野细胞异位导致严重的叶状缺陷,大多数浦肯野细胞聚集在位于中央的簇中。尽管浦肯野细胞迁移严重失败,但在扰乱突变小鼠中,小脑核看起来正常,这表明它们的拓扑结构既不依赖于正常的浦肯野细胞定位,也不依赖于Reelin信号通路。

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