title: Chronological and immunohistochemical observations of cerebellar dysplasia and vermis defect in the hereditary cerebellar vermis defect (CVD) rat.
abstract: Hereditary cerebellar vermis defect (CVD) rats, a new neurological mutant, developed both cerebellar vermis defect and cerebellar dysplasia. Developmental alterations in the cerebellum of the CVD rats were studied chronologically and immunohistochemically. The earliest architectural abnormality was a maldevelopment of the inferior cerebellar peduncle from embryonic day 17 (E17), leading to an indistinct separation between the cerebellum and the pons. From E19, the CVD rats lacked vermis development and, therefore, the cerebellar hemispheres were fused. After birth, Purkinje cells and external granule cells (EGCs) penetrated into the pontine tissue, but retained their normal position until postnatal day 10. Cerebellar lamination began to be disturbed due to abnormal perivascular aggregations of the EGCs, resulting in convoluted and occasionally perivascular lamination. There were no Bergmann glia in the heterotopic cerebellum of the pons, and abnormally arranged Bergmann glia were observed in the mildly disorganized cerebellar hemispheres. Immunohistochemistry for calbindin revealed that abnormal orientation of the Purkinje cells might be related to the perivascular EGCs. Parvalbumin-immunopositive microneurons were seen only in the disarranged molecular layers, and synaptophysin-immunopositive cerebellar glomeruli were present in the afflicted internal granular layers. These findings suggest that perivascular EGCs may play an important role in cerebellar dysplasia and the developmental plasticity in the altered cerebellogenesis.
authors.affiliation: Department of Veterinary Pathology, College of Agriculture, Osaka Prefecture University, Osaka, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kuwamura
authors.forename: A
authors.initials: A
authors.lastname: Ishida
authors.forename: J
authors.initials: J
authors.lastname: Yamate
authors.forename: K
authors.initials: K
authors.lastname: Kato
authors.forename: T
authors.initials: T
authors.lastname: Kotani
authors.forename: S
authors.initials: S
authors.lastname: Sakuma
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D000906:Antibodies / Q000276:immunology; D064026:Calbindins; D002526:Cerebellar Diseases / Q000196:embryology / Q000235:genetics / Q000473:pathology*; D002531:Cerebellum / Q000002:abnormalities* / Q000196:embryology / Q000473:pathology; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000276:immunology; D007150:Immunohistochemistry; D008297:Male; D009457:Neuroglia / Q000473:pathology; D010320:Parvalbumins / Q000276:immunology; D051381:Rats; D011922:Rats, Mutant Strains; D064030:S100 Calcium Binding Protein G / Q000276:immunology; D014746:Vimentin / Q000276:immunology
chemical_list: D000906:Antibodies; D064026:Calbindins; D005904:Glial Fibrillary Acidic Protein; D010320:Parvalbumins; D064030:S100 Calcium Binding Protein G; D014746:Vimentin