Cerebellar granule neurons induce Cyclin D1 before the onset of motor symptoms in Huntington's disease mice.

ABSTRACT: Although Huntington's disease (HD) is classically defined by the selective vulnerability of striatal projection neurons, there is increasing evidence that cerebellar degeneration modulates clinical symptoms. However, little is known about cell type-specific responses of cerebellar neurons in HD. To dissect early disease mechanisms in the cerebellum and cerebrum, we analyzed translatomes of neuronal cell types from both regions in a new HD mouse model. For this, HdhQ200 knock-in mice were backcrossed with the calm 129S4 strain, to constrain experimental noise caused by variable hyperactivity of mice in a C57BL/6 background. Behavioral and neuropathological characterization showed that these S4-HdhQ200 mice had very mild behavioral abnormalities starting around 12 months of age that remained

SUBMITTER: Bauer S 

PROVIDER: S-EPMC9854201 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature