Linked Life Data

7930609

Source:http://linkedlifedata.com/resource/pubmed/id/7930609

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
1994-11-23
pubmed:abstractText
Canine X-linked severe combined immunodeficiency disease (XSCID) is characterized by a failure to thrive, thymic dysplasia, and a lack of a T lymphocyte mitogenic response. As in human XSCID, affected dogs in our colony have a mutation in the IL-2R-gamma gene. This mutation dramatically altered T lymphocyte development, because XSCID thymi were severely reduced in size and cellularity, contained an increased proportion of immature CD4-CD8- thymocytes, a decreased proportion of intermediate CD4+CD8+ thymocytes, and a normal proportion of CD4+CD8- and CD4-CD8+ thymocytes. XSCID thymi were also deficient in the percentage of CD3-L+ thymocytes. Interestingly, several XSCID dogs had normal percentages of CD3-L+ PBL. Although the mutation did not interfere with IL-2 production, PHA-activated XSCID PBL demonstrated severely diminished IL-2 binding and were nonresponsive to IL-2. These results indicate that the lack of a functional IL-2R-gamma chain in dogs with XSCID primarily affects developing CD4-CD8- thymocytes as they acquire the cell surface Ag CD3-L and interferes with the ability of peripheral T lymphocytes to bind and respond to IL-2.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0022-1767
pubmed:author
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
153
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
4006-15
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1994
pubmed:articleTitle
T lymphocyte development and function in dogs with X-linked severe combined immunodeficiency.
pubmed:affiliation
Department of Clinical Studies, School of Veterinary Medicine, University of Pennsylvania, Philadelphia 19104.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.