title: A commentary on the utility of a new L-DOPA-responsive dystonia mouse model.
abstract: In a recent issue of Brain, we reported on the generation and characterization of a mouse model of the rare disease L-DOPA-responsive dystonia (DRD). Here, we discuss the utility of these mice for understanding broader disease processes and treatment strategies. Using specific experimental designs that either work "forward" from genetic etiology or "backward" from the symptomatic presentation, we discuss how our data and future work can be used to understand broader themes.
authors.affiliation: Department of Pharmacology, Emory University School of Medicine , Atlanta, GA, USA.
authors.forename: Samuel J
authors.initials: SJ
authors.lastname: Rose
authors.affiliation: Department of Pharmacology, Emory University School of Medicine, Atlanta, GA, USA; Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Hess
keywords: Basal ganglia; dopamine; neurogenetics; striatum; tyrosine hydroxylase