A comparative study of Purkinje cells in two RORα gene mutant mice: staggerer and RORα−/−
| Autor: | Michael Becker-André, Francesca Capone, Nicole Delhaye-Bouchaud, Florence Frédéric, Jean Mariani, Mohamed Doulazmi |
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| Rok vydání: | 2001 |
| Předmět: |
Male
Cerebellum Purkinje cell Receptors Cytoplasmic and Nuclear Gene Mutant Biology medicine.disease_cause Calbindin Mice Mice Neurologic Mutants Purkinje Cells Developmental Neuroscience medicine Animals Cell Size Mice Knockout Genetics Phenocopy Mutation Nuclear Receptor Subfamily 1 Group F Member 1 Molecular biology Mice Inbred C57BL Retinoic acid receptor Phenotype medicine.anatomical_structure Nuclear receptor Trans-Activators Female Developmental Biology |
| Zdroj: | Developmental Brain Research. 127:165-174 |
| ISSN: | 0165-3806 |
| Popis: | The staggerer (Rora(sg/sg)) mutation is a deletion in the RORalpha gene, one member of a family of nuclear receptor genes related to the retinoic acid receptor. Recently Steinmayr et al. (Proc. Natl. Acad. Sci. USA 95 (1998) 3960) generated a RORalpha null-mutant mouse (Rora(-/-)) by using a targeting vector in which a beta-Gal gene replaces the second finger of the DNA-binding domain of RORalpha. The Rora(-/-) cerebellum is qualitatively a phenocopy of the Rora(sg/sg) one, but the two strains differ slightly in their motor skills. To address the question whether the morphological defects in the Rora(-/-) cerebellum are identical to the Rora(sg/sg) one, we compared number and size of Purkinje cells in both staggerer and RORalpha null-mutant mice, using calbindin (CaBP) immunohistochemistry and revelation of beta-Gal activity. Compared to control cerebella the Rora(sg/sg) cerebellum has 82% fewer CaBP-positive cells. In Rora(-/-) mouse, all the the beta-Gal-positive Purkinje cells also expressed CaBP, but the cerebellum contained 78% less CaBP-positive cells than control, a deficit not different from the one observed in Rora(sg/sg). We show similar mediolateral compartments in Purkinje cell number and cytological abnormality in Rora(sg/sg) and Rora(-/-) mice. These results provide quantitative support for the hypothesis that the cerebellar phenotype in the homozygous Rora(sg/sg) is due to the lack of function of the RORalpha gene. |
| Databáze: | OpenAIRE |
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