Linked Life Data

10797391

Source:http://linkedlifedata.com/resource/pubmed/id/10797391

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
2000-5-19
pubmed:abstractText
The purpose of this study was to further characterize neuromyotonia in mice with deletions and point mutations of myelin protein genes. Clinical observation showed irregular stretching of the hindlimbs, tremor and generalized myokymia in mice with targeted deletions of the genes encoding myelin protein zero (P0-/-) or peripheral myelin protein 22 (Pmp22-/-), and Trembler mice, which carry a point mutation of Pmp22. By electromyography (EMG), we found irregular high-frequency bursts of spontaneous motor unit activity and rhythmic doublet or multiplet discharges of motor units in these mouse models of human hereditary neuropathies. The EMG signs are typical for neuromyotonia and myokymia, respectively. The activity persisted after a proximal nerve section in many cases, localizing the generator to the peripheral nerve or the muscle. We now show that blocking neuromuscular transmission with suxamethonium abolished the spontaneous activity, ruling out a muscle origin. Phenytoin ameliorated the motor behavior. Taken together, our study shows that neuromyotonia develops in different mouse models of hereditary myelinopathies. This indicates that spontaneous motor unit activity may underlie neuromyotonia, which is occasionally observed in Charcot-Marie-Tooth disease. These animal models will be useful to study the pathogenesis of neuromyotonia.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0148-639X
pubmed:author
pubmed:copyrightInfo
Copyright 2000 John Wiley & Sons, Inc.
pubmed:issnType
Print
pubmed:volume
23
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
696-701
pubmed:dateRevised
2009-11-19
pubmed:meshHeading
pubmed:year
2000
pubmed:articleTitle
Neuromyotonia in mice with hereditary myelinopathies.
pubmed:affiliation
Department of Neurology, Julius-Maximilians-University, Josef-Schneider-Str. 11, D-97080 Würzburg, Germany. NEUK152@mail.uni-wuerzburg.de
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't