Mouse models of neurodevelopmental disease of the basal ganglia and associated circuits

Samuel S. Pappas, Daniel K. Leventhal, Roger L. Albin, William T. Dauer

Research output: Chapter in Book/Report/Conference proceedingChapter

25 Scopus citations

Abstract

This chapter focuses on neurodevelopmental diseases that are tightly linked to abnormal function of the striatum and connected structures. We begin with an overview of three representative diseases in which striatal dysfunction plays a key role-Tourette syndrome and obsessive-compulsive disorder, Rett's syndrome, and primary dystonia. These diseases highlight distinct etiologies that disrupt striatal integrity and function during development, and showcase the varied clinical manifestations of striatal dysfunction. We then review striatal organization and function, including evidence for striatal roles in online motor control/action selection, reinforcement learning, habit formation, and action sequencing. A key barrier to progress has been the relative lack of animal models of these diseases, though recently there has been considerable progress. We review these efforts, including their relative merits providing insight into disease pathogenesis, disease symptomatology, and basal ganglia function.

Original languageEnglish (US)
Title of host publicationCurrent Topics in Developmental Biology
PublisherAcademic Press Inc.
Pages97-169
Number of pages73
DOIs
StatePublished - 2014
Externally publishedYes

Publication series

NameCurrent Topics in Developmental Biology
Volume109
ISSN (Print)0070-2153

Keywords

  • Basal ganglia
  • Development
  • Disease
  • Dystonia
  • Mouse model
  • OCD
  • Rett syndrome
  • Striatum
  • Tourette

ASJC Scopus subject areas

  • Developmental Biology
  • Cell Biology

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