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Paroxysmal dyskinesias in mice
Author(s) -
Shirley Thomas L.,
Rao Lekha M.,
Hess Ellen J.,
Jinnah H. A.
Publication year - 2008
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21829
Subject(s) - paroxysmal dyskinesia , dyskinesia , neuroscience , pathophysiology , neurological disorder , epilepsy , channelopathy , movement disorders , psychology , animal model , disease , medicine , central nervous system disease , parkinson's disease , pathology
Animal models of human disease are important tools for revealing the underlying mechanisms of pathophysiology and developing therapeutic strategies. Several unique mouse calcium channel mutants have been identified with nonepileptic, episodic dyskinetic movements that are phenotypically similar to human paroxysmal dyskinesias. In this report, video demonstrations of these motor attacks are provided for two previously described mouse mutants, tottering and lethargic, as well as a new one, rocker. Semiquantitative comparisons using two different rating scales reveal differences in attack morphology, severity, and duration among the strains. These mice provide three independent models of paroxysmal dyskinesia and support for prior proposals that channelopathies may underlie the human disorders. © 2007 Movement Disorder Society