Source:http://linkedlifedata.com/resource/pubmed/id/18336930
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
2
|
| pubmed:dateCreated |
2008-4-4
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| pubmed:abstractText |
Arylsulfatase A (ASA)-deficient mice represent an animal model for the lysosomal storage disorder metachromatic leukodystrophy (MLD). Although the model has been applied in pathophysiological and therapeutic studies, the behavioural phenotype of ASA(-/-) mice is only partially characterized, and the most decisive outcome measures for therapy evaluation only emerge beyond 1 year of age. Presently, ASA(-/-) mice and ASA(+/-) control mice were studied at 6 and 12 months of age on an extensive battery including tests of neuromotor ability, exploratory behaviour, and learning and memory. Overt signs of ataxia were not observed in 6-month-old ASA(-/-) mice, but quantitative gait analysis during open-field exploration revealed that ASA(-/-) mice displayed increased hind base width and increased stride lengths for all paws. Their covert motor incoordination was evident in a correlation analysis which unveiled decreased harmonisation of concurrent gait parameters. For example, while ASA(+/-) controls demonstrated substantial convergence of front and hind base width (r=0.54), these variables actually diverged in ASA(-/-) mice (r=-0.37). Furthermore, various behavioural observations indicated emotional alterations in ASA(-/-) mice. Six-month-old ASA(-/-) mice also showed decreased response rates in scheduled operant responding. The present findings could provide relevant behavioural outcome measures for further use of this murine MLD model in preclinical studies.
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| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
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| pubmed:month |
Jun
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| pubmed:issn |
0166-4328
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| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
3
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| pubmed:volume |
189
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| pubmed:owner |
NLM
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| pubmed:authorsComplete |
Y
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| pubmed:pagination |
306-16
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| pubmed:meshHeading |
pubmed-meshheading:18336930-Age Factors,
pubmed-meshheading:18336930-Animals,
pubmed-meshheading:18336930-Avoidance Learning,
pubmed-meshheading:18336930-Cerebellum,
pubmed-meshheading:18336930-Cerebroside-Sulfatase,
pubmed-meshheading:18336930-Cerebrum,
pubmed-meshheading:18336930-Conditioning, Operant,
pubmed-meshheading:18336930-Disease Models, Animal,
pubmed-meshheading:18336930-Exploratory Behavior,
pubmed-meshheading:18336930-Female,
pubmed-meshheading:18336930-Gait,
pubmed-meshheading:18336930-Gait Ataxia,
pubmed-meshheading:18336930-Inhibition (Psychology),
pubmed-meshheading:18336930-Leukodystrophy, Metachromatic,
pubmed-meshheading:18336930-Lipidoses,
pubmed-meshheading:18336930-Male,
pubmed-meshheading:18336930-Matched-Pair Analysis,
pubmed-meshheading:18336930-Mice,
pubmed-meshheading:18336930-Mice, Inbred C57BL,
pubmed-meshheading:18336930-Mice, Knockout,
pubmed-meshheading:18336930-Motor Activity,
pubmed-meshheading:18336930-Phenotype,
pubmed-meshheading:18336930-Psychomotor Performance
|
| pubmed:year |
2008
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| pubmed:articleTitle |
Early signs of neurolipidosis-related behavioural alterations in a murine model of metachromatic leukodystrophy.
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| pubmed:affiliation |
Laboratory of Biological Psychology, Department of Psychology, University of Leuven, Leuven, Belgium.
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| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|