Models of dystonia: an update

P. Imbriani, G. Ponterio, A. Tassone, G. Sciamanna, I. El Atiallah, P. Bonsi, A. Pisani

Research output: Contribution to journalReview articlepeer-review

Abstract

Although dystonia represents the third most common movement disorder, its pathophysiology remains still poorly understood. In the past two decades, multiple models have been generated, improving our knowledge on the molecular and cellular bases of this heterogeneous group of movement disorders. In this short survey, we will focus on recently generated novel models of DYT1 dystonia, the most common form of genetic, “isolated” dystonia. These models clearly indicate the existence of multiple signaling pathways affected by the protein mutation causative of DYT1 dystonia, torsinA, paving the way for potentially multiple, novel targets for pharmacological intervention.

Original languageEnglish
Article number108728
JournalJournal of Neuroscience Methods
Volume339
DOIs
Publication statusPublished - Jun 1 2020

Keywords

  • acetylcholine
  • dopamine
  • dystonia
  • DYT1
  • interneurons
  • movement disorders
  • striatum

ASJC Scopus subject areas

  • Neuroscience(all)

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