Cerebellar defect and impaired motor coordination in mice lacking vimentin.
Vimentin belongs to the family of intermediate filament (IF) proteins. During the nervous system development in mammals, it is transiently expressed in precursor cells of neuronal and glial lineages, and then it is progressively replaced by other types of IF proteins. Surprisingly, mice knock-out for vimentin develop and reproduce without any apparent defects (Colucci-Guyon et al. Cell 79:679-694, 1994). In adult rodents, Bergmann glia (BG) of the cerebellum continue to express vimentin together with glial fibrillary acidic protein (GFAP). A careful analysis of cerebellar morphology and ultrastructure in mutants showed poorly developed and highly abnormal BG, whereas the migration of granular neurons proceeded normally. Moreover, many Purkinje cells (PC) appeared stunted with a loss of spiny branchlets, and some of them were necrotic. Finally, impaired motor coordination was evidenced by behavioral tests. These observations demonstrate a role for vimentin in contributing to the normal development and morphology of BG and reveal a hitherto unreported functional relationship between BG and PC.
Colucci-Guyon
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E
Unité de Biologie du Développement, Institut Pasteur, Paris, France.
Giménez Y Ribotta
M
M
Maurice
T
T
Babinet
C
C
Privat
A
A
D000818:Animals; D001522:Behavior, Animal / Q000187:drug effects; D002526:Cerebellar Diseases / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D005106:Exploratory Behavior / Q000187:drug effects; D005904:Glial Fibrillary Acidic Protein / Q000096:biosynthesis; D018782:Maze Learning / Q000187:drug effects; D051379:Mice; D018345:Mice, Knockout; D009457:Neuroglia / Q000187:drug effects / Q000502:physiology / Q000648:ultrastructure; D009474:Neurons / Q000378:metabolism / Q000502:physiology / Q000648:ultrastructure; D004856:Postural Balance / Q000187:drug effects; D011597:Psychomotor Performance / Q000502:physiology*; D011689:Purkinje Cells / Q000187:drug effects / Q000502:physiology / Q000648:ultrastructure; D014746:Vimentin / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism
D005904:Glial Fibrillary Acidic Protein; D014746:Vimentin