MovDisordV3, Main, Exploration, bibRecord, 004A48

Investigation of the role of the cerebellum in the myoclonic‐like movement disorder exhibited by tottering mice

Identifieur interne : 004A48 ( Main/Exploration ); précédent : 004A47; suivant : 004A49

Investigation of the role of the cerebellum in the myoclonic‐like movement disorder exhibited by tottering mice

Auteurs : Louise C. Abbott [États-Unis] ; Melissa Bump [États-Unis] ; Amy Brandl [États-Unis] ; Scarlett De Laune [États-Unis]

Source :

Movement Disorders [ 0885-3185 ] ; 2000-09.

RBID : ISTEX:72A799D0597F6754FDFC2DAA68C02CA2EE329197

Descripteurs français

Pascal (Inist)
- Animal transgénique, Calcium, Canal ionique, Cervelet, Etiopathogénie, Gène, Modèle animal, Mutation, Myoclonie, Souris.
Wicri :
- topic : Animal transgénique, Calcium.

English descriptors

KwdEn :
- Animal model, Animals, Brain Mapping, Calcium, Calcium Channels (physiology), Calcium Channels, N-Type, Calcium Channels, P-Type, Calcium Channels, Q-Type, Calcium ion channel, Cerebellar Cortex (physiopathology), Cerebellar Nuclei (physiopathology), Cerebellar cortical lesion, Cerebellum, Cerebellum (physiopathology), Climbing fiber, Deep cerebellar nuclei, Epilepsies, Myoclonic (physiopathology), Etiopathogenesis, Female, Gene, Granule cell, Ionic channel, Male, Mice, Mice, Inbred C57BL, Mice, Neurologic Mutants, Mouse, Mutation, Myoclonus, Myoclonus (physiopathology), Norepinephrine (physiology), Parallel fiber, Purkinje Cells (physiology), Purkinje cell, Transgenic animal.
MESH :
- chemical , physiology : Calcium Channels, Norepinephrine.
- physiology : Purkinje Cells.
- physiopathology : Cerebellar Cortex, Cerebellar Nuclei, Cerebellum, Epilepsies, Myoclonic, Myoclonus.
- Animals, Brain Mapping, Calcium Channels, N-Type, Calcium Channels, P-Type, Calcium Channels, Q-Type, Female, Male, Mice, Mice, Inbred C57BL, Mice, Neurologic Mutants.

Abstract

Recently it has been discovered that defects in neuronal ion channels can result in seizure disorders. The tottering mouse is a genetic animal model carrying a mutation in the α1A calcium channel subunit that causes these mice to exhibit generalized petit mal‐like epilepsy, cerebellar ataxia, and an intermittent movement disorder that has some characteristics similar to myoclonus or myoclonic epilepsy. We postulate that abnormal cerebellar Purkinje cell output to the deep cerebellar nuclei results in the intermittent movement disorder observed in these mice. The frequency and duration of seizure activity were measured in tottering mice before and 2 weeks after surgical or chemical lesioning of the cerebellum. Surgical lesions in the anterior cerebellar vermis of tottering mice produced significant reductions in seizure duration and frequency. Surgical lesioning of the posterior cerebellar vermis had no significant effect. Chemical lesions of the same cerebellar regions, using a locally applied neurotoxin, NMD‐L‐A, appear to produce effects similar to the surgical lesions. These data indicate that anterior vermal cerebellar output is important for production of the seizures associated with the intermittent movement disorder observed in tottering mice.

Url:

https://api.istex.fr/document/72A799D0597F6754FDFC2DAA68C02CA2EE329197/fulltext/pdf

DOI: 10.1002/mds.870150710

Affiliations:

Le document en format XML

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<front><div type="abstract" xml:lang="en">Recently it has been discovered that defects in neuronal ion channels can result in seizure disorders. The tottering mouse is a genetic animal model carrying a mutation in the α1A calcium channel subunit that causes these mice to exhibit generalized petit mal‐like epilepsy, cerebellar ataxia, and an intermittent movement disorder that has some characteristics similar to myoclonus or myoclonic epilepsy. We postulate that abnormal cerebellar Purkinje cell output to the deep cerebellar nuclei results in the intermittent movement disorder observed in these mice. The frequency and duration of seizure activity were measured in tottering mice before and 2 weeks after surgical or chemical lesioning of the cerebellum. Surgical lesions in the anterior cerebellar vermis of tottering mice produced significant reductions in seizure duration and frequency. Surgical lesioning of the posterior cerebellar vermis had no significant effect. Chemical lesions of the same cerebellar regions, using a locally applied neurotoxin, NMD‐L‐A, appear to produce effects similar to the surgical lesions. These data indicate that anterior vermal cerebellar output is important for production of the seizures associated with the intermittent movement disorder observed in tottering mice.</div>
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Movement Disorders (revue)

Investigation of the role of the cerebellum in the myoclonic‐like movement disorder exhibited by tottering mice

Investigation of the role of the cerebellum in the myoclonic‐like movement disorder exhibited by tottering mice

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

	Movement Disorders (revue)
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