title: Progressive hind limb paralysis in mice carrying a v-Mos transgene.
abstract: To study the function of the protooncogene Mos in mouse brain development we have created a transgenic mouse model system in which an activated form of the gene, the murine retroviral v-Mos gene, is highly overexpressed in the brain. Six transgenic founder animals and mice of one established transgenic line (line TG66) displayed a progressive hind limb paralysis with onset between 18 days and 9 months. The severity of the neurological phenotype correlated with pathological alterations and the degree of v-Mos expression in the brain which varied between individual animals of line TG66. The most striking feature of the brain pathology was the presence of large, abnormal astrocytes in the cerebellum, medulla, thalamus and in the dorsal horn of the spinal cord. These areas also contained shrunken and basophilic neurons whose cytoplasm was abnormally immunoreactive for phosphorylated epitopes of neurofilaments. In addition to neuropathologic changes, these mice also displayed aberrant eye lens differentiation and absence of hair cells in the inner ear. These results establish v-Mos transgenic mice as a model system to study progressive neurodegenerative disease and provide further evidence that the Mos protein-serine/threonine kinase has a function in brain development.
authors.affiliation: Ludwig Institute for Cancer Research, St. Mary's Hospital Medical School, London, United Kingdom.
authors.forename: F
authors.initials: F
authors.lastname: Propst
authors.forename: L C
authors.initials: LC
authors.lastname: Cork
authors.forename: R M
authors.initials: RM
authors.lastname: Kovatch
authors.forename: A B
authors.initials: AB
authors.lastname: Kasenally
authors.forename: R
authors.initials: R
authors.lastname: Wallace
authors.forename: M P
authors.initials: MP
authors.lastname: Rosenberg
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism; D002490:Central Nervous System / Q000473:pathology; D016258:Genes, mos; D006614:Hindlimb; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics*; D010243:Paralysis / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D010641:Phenotype; D012313:RNA / Q000378:metabolism
chemical_list: D012313:RNA