Figure 2

Development in cerebellar neuron conditional dystroglycan-null mice. Macroscopic images of cerebellar morphology and hematoxylin-eosin stained, mid-sagittal sections of adult cerebella from control (A, B), malpha6-Cre/DG-null (D, E), and PCP2-Cre/DG-null (G, H) mice. Higher magnification of PCP2-Cre/DG-null mid-sagittal cerebellar section showing small heterotopia (C, F). Immunofluorescent staining of α-dystroglycan (αDG) and glial fibrillary acidic protein (GFAP) (both green) in control (I, J) and PCP2-Cre/DG-null cerebella (L, M). Double staining of laminin (LM; green) and neuronal nuclear protein (NeuN; red) in control (K) and PCP2-Cre/DG-null (N) cerebella. The cerebellum of both malpha6-Cre/ and PCP2-Cre/DG-null develop normally without gross malformations. Dystroglycan is expressed as puncta in Purkinje cells (double daggers; ‡) and concentrated at glial endfeet (carets; ^) as well as blood vessels (right double angle brackets; ») in adult control cerebellum (I). In the PCP2-Cre/DG-null mice, dystroglycan expression is greatly reduced in Purkinje cells, but its expression in glial cells remains unaltered at the glia limitans and surrounding vessels (L). Loss of dystroglycan expression in the PCP2-Cre/DG-null does not affect Bergmann glia morphology (M) while causes in a mild degree of GC ectopia (asterisks; *) immediately beneath an intact glia limitans (carets; ^) (N). DAPI (blue) was used as a nuclear counter stain. Scale bar: 20 μm.