Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1996-4-26
pubmed:abstractText
It has been suggested that the overexpression of copper-zinc superoxide dismutase (SOD-1) in Down's syndrome (DS) patients may be involved in expression of some of the phenotypic characteristics observed in these patients. To explore the possible role of SOD-1 overexpression in the premature thymic involution and immunologic disorders observed in DS patients, transgenic mice overexpressing the human SOD-1 gene have been generated and their thymuses have been studied at the ultrastructural level. Our observations show premature involution of the thymus in SOD-1 transgenic mice, with a strong modification of the thymic microenvironment starting at approximately 3-4 months of age. The thymic microenvironment in 7-month-old transgenic mice is similar to that observed in 20-month-old control mice. We suggest that these results are consistent with the role of SOD-1 overexpression in the early thymic involution observed in DS patients. These transgenic mice provide an interesting model to investigate the deleterious effect of increased dosage of some chromosome 21 genes such as SOD-1 in the pathogenesis of DS.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0023-6837
pubmed:author
pubmed:issnType
Print
pubmed:volume
74
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
617-26
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1996
pubmed:articleTitle
Transgenic mice overexpressing the human Cu/Zn-SOD gene: ultrastructural studies of a premature thymic involution model of Down's syndrome (trisomy 21).
pubmed:affiliation
U.345 INSERM, Hôpital Necker, Paris, France.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't