Linked Life Data

17549011

Source:http://linkedlifedata.com/resource/pubmed/id/17549011

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2007-6-5
pubmed:abstractText
Human familial amyotrophic lateral sclerosis with an H46R mutant Cu/Zn superoxide dismutase (SOD1) gene is characterized by initial muscle weakness and atrophy in the legs and a very long-term clinical course (approximately 15 years). Transgenic mice with this mutation generated in our laboratory occasionally showed aggregates in the anterior horns and axonal degeneration in all white matter sections of the spinal cord on plastic sections at the presymptomatic stages (12 and 16 weeks old), although conventional staining revealed no pathologic changes. At the symptomatic stages (20 and 24 weeks), loss of anterior horn neurons was observed. On plastic sections, aggregates were frequently seen not only in the anterior horns but also in the posterior horns and in all sections of white matter. Degenerated fibers were observed in the anterior and posterior roots as well as in white matter. Electron and immunoelectron microscopic observation revealed human SOD1- and ubiquitin-positive aggregates consisting of intermediate filaments in the anterior horn even from an early presymptomatic stage. Thus, H46R mutant SOD1 transgenic mice are characterized by widespread pathologic changes of the spinal cord that extend beyond the motor system, including many aggregates lacking vacuoles. The close pathologic similarity makes this animal model suitable for the investigation of human familial amyotrophic lateral sclerosis with the mutation.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
0022-3069
pubmed:author
pubmed:issnType
Print
pubmed:volume
66
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
517-24
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
Motor neuron disease in transgenic mice with an H46R mutant SOD1 gene.
pubmed:affiliation
Department of Neurology, Neurological Institute, Tokyo Women's Medical University, Tokyo, Japan.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't