Genetic models for connective tissue disease, scarring and fibrosis

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When vital organs are damaged in disease this results in the development of scar tissue. When the scar is excessive or disporportionate this can itself worsen organ function, a process termed fibrosis. Fibrosis is a result of altered or abnormal healing processes and is a common factor in many common diseases. Less common disorders such as scleroderma (also called systemic sclerosis) cause fibrois in multiple organs including the skin, lungs, heart and blood vessels. We have developed, for the first time, models of fibrotic disease that can be used to study the development and consequences of fibrosis in the skin and internal organs. Through understanding these models, it is very likely that better methods for assessment and treating fibrosis will emerge.