Dysregulation of dopamine receptor D2 as a sensitive measure for Huntington disease pathology in model mice.

ABSTRACT: The ability to quantitatively evaluate the impact of a potential therapeutic intervention for Huntington disease (HD) in animal models for the disease is a critical step in the pathway to development of an effective therapy for this devastating neurodegenerative disorder. We report here an approach that combines a cell-based assay's quantitative accuracy and direct relationship to molecular processes with the ability to directly monitor effects in HD model mouse neurons. To accomplish this goal, we have developed an accurate quantitative reporter assay for a transcript known to be down-regulated as an early consequence of mutant huntingtin expression. This system uses mouse strains carrying a GFP reporter for the expression of the dopamine receptor D2, expressed in the medium spiny neurons

SUBMITTER: Crook ZR 

PROVIDER: S-EPMC3358846 | biostudies-literature | 2012 May

REPOSITORIES: biostudies-literature