Chung S-H, Marzban H, Croci L, Consalez G G, Hawkes R
Department of Cell Biology and Anatomy, Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.
Neuroscience. 2008 May 15;153(3):721-32. doi: 10.1016/j.neuroscience.2008.01.090. Epub 2008 Mar 6.
The mammalian cerebellar cortex is highly compartmentalized. First, it is subdivided into four transverse expression domains: the anterior zone (AZ), the central zone (CZ), the posterior zone (PZ), and the nodular zone (NZ). Within each zone, the cortex is further subdivided into a symmetrical array of parasagittal stripes. The most extensively studied compartmentation antigen is zebrin II/aldolase c, which is expressed by a subset of Purkinje cells forming parasagittal stripes. Stripe phenotypes are specified early in cerebellar development, in part through the action of early B-cell factor 2 (Ebf2), a member of the atypical helix-loop-helix transcription factor family Collier/Olf1/EBF. In the murine cerebellum, Ebf2 expression is restricted to the zebrin II-immunonegative (zebrin II-) Purkinje cell population. We have identified multiple cerebellar defects in the Ebf2 null mouse involving a combination of selective Purkinje cell death and ectopic expression of multiple genes normally restricted to the zebrin II- subset. The nature of the cerebellar defect in the Ebf2 null is different in each transverse zone. In contrast to the ectopic expression of genes characteristic of the zebrin II+ Purkinje cell phenotype, phospholipase Cbeta4 expression, restricted to zebrin II- Purkinje cells in control mice, is well maintained, and the normal number of stripes is present. Taken together, these data suggest that Ebf2 regulates the expression of genes associated with the zebrin II+ Purkinje cell phenotype and that the zebrin II- Purkinje cell subtype is specified independently.
哺乳动物的小脑皮质高度分区。首先，它被细分为四个横向表达域：前区（AZ）、中区（CZ）、后区（PZ）和小结区（NZ）。在每个区域内，皮质进一步细分为一系列对称的矢状旁条纹。研究最广泛的分区抗原是zebrin II/醛缩酶c，它由形成矢状旁条纹的一部分浦肯野细胞表达。条纹表型在小脑发育早期就已确定，部分是通过早期B细胞因子2（Ebf2）的作用，Ebf2是非典型螺旋-环-螺旋转录因子家族Collier/Olf1/EBF的成员。在小鼠小脑中，Ebf2的表达仅限于zebrin II免疫阴性（zebrin II-）的浦肯野细胞群体。我们在Ebf2基因敲除小鼠中发现了多种小脑缺陷，包括选择性浦肯野细胞死亡和多个通常仅限于zebrin II-亚群的基因的异位表达。Ebf2基因敲除小鼠中小脑缺陷的性质在每个横向区域都有所不同。与zebrin II+浦肯野细胞表型特征性基因的异位表达相反，磷脂酶Cβ4的表达（在对照小鼠中仅限于zebrin II-浦肯野细胞）得到很好的维持，并且条纹数量正常。综上所述，这些数据表明Ebf2调节与zebrin II+浦肯野细胞表型相关的基因表达，并且zebrin II-浦肯野细胞亚型是独立确定的。
