Linked Life Data

15344604

Source:http://linkedlifedata.com/resource/pubmed/id/15344604

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3 Suppl 33
pubmed:dateCreated
2004-9-3
pubmed:abstractText
The search for an animal model of systemic sclerosis (SSc) was tenaciously pursued by E.C. LeRoy. We studied several aspects of the tight skin mouse (Tsk) genetics and pathogenesis under his stimulating influence that contributed to a better understanding of the fibrotic scleroderma-like phenotype of this mouse. The identification of the fibrillin-1 mutation in the Tsk mouse and the characterization of the cellular and molecular pathways leading to Tsk fibrosis by numerous research groups has opened new avenues in the investigation of human SSc. The enigmatic connections between autoimmunity and ECM homeostasis in fibrotic diseases have received extensive attention in this mouse in which a prirmary alteration of a connective tissue microfibrilar protein leads to the reproduction of cellular and autoimmune abnormalities strikingly similar to human SSc. The use of this mouse as a tool to explore anti-fibrotic therapeutic interventions has demonstrated its value in providing useful information on the search for a therapy for this untreatable facet of human disease.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0392-856X
pubmed:author
pubmed:issnType
Print
pubmed:volume
22
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
S81-5
pubmed:meshHeading
pubmed:articleTitle
The tight skin mouse: an animal model of systemic sclerosis.
pubmed:affiliation
Servicio de Reumatología and Unidad de Investigación, Hospital 12 de Octubre, Madrid, Spain. jlpablos@h12o.es
pubmed:publicationType
Journal Article