Movement Disorders (revue)

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Paroxysmal Dyskinesias in Mice

Identifieur interne : 000020 ( Pmc/Curation ); précédent : 000019; suivant : 000021

Paroxysmal Dyskinesias in Mice

Auteurs : Thomas L. Shirley ; Lekha M. Rao ; Ellen J. Hess ; H. A. Jinnah

Source :

RBID : PMC:2887756

Abstract

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.


Url:
DOI: 10.1002/mds.21829
PubMed: 17999434
PubMed Central: 2887756

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PMC:2887756

Le document en format XML

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<p id="P1">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.</p>
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<surname>Rao</surname>
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<degrees>MD</degrees>
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<given-names>Ellen J.</given-names>
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<aff id="A1">Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA</aff>
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Correspondence to: H.A. Jinnah, Meyer 6-181, Johns Hopkins University, Baltimore, MD 21287. E-mail:
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<volume>23</volume>
<issue>2</issue>
<fpage>259</fpage>
<lpage>264</lpage>
<abstract>
<p id="P1">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.</p>
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