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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
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pubmed:dateCreated |
1980-12-18
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pubmed:abstractText |
A new inherited neurological disease in the mouse (the mutant twitcher) has been studied. Transmission is by an autosomal recessive gene (twi). Affected animals are apparently normal at birth but develop a generalized tremor at about 3 weeks of age followed by progressive weakness and wasting. The disease is fatal by 3 months. The principal pathological changes affect the myelin of both central and peripheral nervous systems. Degeneration of myelin sheaths and the presence of multinucleated macrophages with PAS-positive cytoplasm are characteristic findings. Peripheral nerves show remyelination following demyelination. Electron microscopically the macrophages contain a variety of inclusions in which there are crystalline and multi-angular structures and twisted tubules. The abnormalities closely resemble those found in globoid cell leucodystrophy (Krabbe's disease) in man.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0006-8950
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
103
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
695-710
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pubmed:dateRevised |
2010-11-18
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pubmed:meshHeading |
pubmed-meshheading:7417782-Animals,
pubmed-meshheading:7417782-Brain,
pubmed-meshheading:7417782-Disease Models, Animal,
pubmed-meshheading:7417782-Genetic Linkage,
pubmed-meshheading:7417782-Leukodystrophy, Globoid Cell,
pubmed-meshheading:7417782-Mice,
pubmed-meshheading:7417782-Mice, Neurologic Mutants,
pubmed-meshheading:7417782-Peripheral Nerves
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pubmed:year |
1980
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pubmed:articleTitle |
Hereditary leucodystrophy in the mouse: the new mutant twitcher.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.
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