Animal Models Of Scleroderma And Their Application Facilities

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Laboratorinė medicina. 2014,
t. 16,
Nr. 4,
p. 187 -
193

Relevant animal models are essential tools to investigate in depth the patho-genesis of systemic sclerosis and search of new therapeutic interventions. Several models of scleroderma are currently available but, unfortunately, none of these reflects all features of the human disease. Some of these models present inflammation followed by fibro-sis, whether some others primarily mimic autonomous fibroblast activation. Only few models are available and suitable to study small vessels vasculo-pathy. Hence, we provide an overview of the most import ant models of systemic sclerosis. There are bleomycin-, hypochlorous acid-, vinil chloride-, topoisomerase I and complete Freund's adjuvant-in duced sys temic sclero sis mod els, sclero dermatous chronic graft-versus-host mice disease model, with prevailing features of inflammation, autoimmunity and fibrosis. Recently three new inducible animal models have been revealed: reactive oxygen species-, angiotensin II- and topoisome-rase I and complete Freund's adjuvant-induced scleroderma models. Transgenic, spontaneous, and knockout gene mice models of scleroderma also widely used. Only some models such as UCD-200 ir UCD-206 chickens model, ar Fra-2 mice model are relevant for investigation of small vascular damage. The choise of experimental model of systemic sclerosis depends on the aim of investigation. Drugs that fulfil evidence level A should be tested in at least two of the animal models mentioned earlier addressing different aspects of the disease. Moreover, the therapeutic effect should be shown in different organs involved in systemic sclerosis. In this article we discuss about widely used animal models of systemic sclerosis and its application facilities for the search of therapeutic tools.

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