title: Gender effect on Purkinje cell loss in the cerebellum of the heterozygous reeler mouse.
abstract: Homozygous mutant mice such as staggerer (sg/sg) or reeler (rl/rl) exhibit a marked ataxia associated with an atrophic cerebellum during the first postnatal weeks and a reduced number of Purkinje cells, the deficit reaching about 75% in sg/sg and 50% in rl/rl as compared to age- and sex-matched mice from the same strain background. These two mutations are classically viewed as recessive, but we have recently shown that heterozygous staggerer (+/sg) mice exhibit a progressive and age-related loss of Purkinje cells between 3 and 12 months of age, despite their apparent clinical normality (Shojaeian-Zanjani et al., 1992). In the present study, we have investigated whether a similar cell loss exists in the cerebellum of heterozygous +/rl mice. The number of Purkinje cells was counted in serial parasagittal sections of the cerebellum of +/rl and their +/+ littermates at 3, 16 and 26 months of age. Our results reveal a 16% deficit in the number of Purkinje cells in 3-month-old +/rl and a 24% one in 16-month-old animals: surprisingly this deficit is only present in the +/rl males, while the females are spared. These results suggest that the reeler gene (D'Arcangelo et al., 1995) exerts its effect on Purkinje cell number in a gender-specific fashion in heterozygous mutant mice.
authors.affiliation: Laboratoire de Neurobiologie du Développement, Institut des Neurosciences (CNRS URA 1488), Université Pierre et Marie Curie, Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: Hadj-Sahraoui
authors.forename: F
authors.initials: F
authors.lastname: Frédéric
authors.forename: N
authors.initials: N
authors.lastname: Delhaye-Bouchaud
authors.forename: J
authors.initials: J
authors.lastname: Mariani
mesh_terms: D000375:Aging; D000818:Animals; D002524:Cerebellar Ataxia / Q000235:genetics*; D002531:Cerebellum / Q000166:cytology / Q000254:growth & development*; D005260:Female; D006579:Heterozygote; D008297:Male; D051379:Mice; D008818:Mice, Neurologic Mutants; D011689:Purkinje Cells / Q000166:cytology*; D012727:Sex Characteristics