Paroxysmal dyskinesias in the lethargic mouse mutant

Zubair Khan, H. A. Jinnah

Research output: Contribution to journalArticlepeer-review

Abstract

Lethargic mutant mice carry a mutation in the CCHB4 gene, which encodes the β4 subunit of voltage-regulated calcium channels. These mutants have been shown to display a complex neurobehavioral phenotype that includes EEG discharges suggestive of absence epilepsy, chronic ataxia, and hypoactivity. The current studies demonstrate a fourth element of their phenotype, consisting of transient attacks of severe dyskinetic motor behavior. These attacks can be triggered by specific environmental and chemical influences, particularly those that stimulate locomotor activity. Behavioral and EEG analyses indicate that the attacks do not reflect motor epilepsy, but instead resemble a paroxysmal dyskinesia. The lethargic mutants provide additional evidence that calcium channelopathies can produce paroxysmal dyskinesias and provide a novel model for studying this unusual movement disorder.

Original languageEnglish (US)
Pages (from-to)8193-8200
Number of pages8
JournalJournal of Neuroscience
Volume22
Issue number18
DOIs
StatePublished - Sep 15 2002

Keywords

  • Calcium channel
  • Channelopathy
  • Epilepsy
  • Lethargic
  • Mouse mutant
  • Movement disorder
  • Paroxysmal dyskinesia

ASJC Scopus subject areas

  • Neuroscience(all)

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