[PDF][PDF] The tight skin mouse: an animal model of systemic sclerosis

JL Pablos, ET Everett, JS Norris - Clinical and experimental …, 2004 - researchgate.net
Clinical and experimental rheumatology, 2004researchgate.net
The search for an animal model of systemic sclerosis (SSc) was tenaciously pursued by EC
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 …
Abstract
The search for an animal model of systemic sclerosis (SSc) was tenaciously pursued by EC 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 autoinmunity and ECM homeostasis in fibrotic diseases have received extensive attention in this mouse in which a primary 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.
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