Genetic models for connective tissue disease, scarring and fibrosis

当重要器官在疾病中受损时，这会导致疤痕组织的发展。当疤痕过多或不稳定时，这本身会恶化器官功能，这一过程称为纤维化。纤维化是愈合过程改变或异常的结果，是许多常见疾病的共同因素。不太常见的疾病，如硬皮病（也称为系统性硬化症），导致纤维在多个器官，包括皮肤，肺，心脏和血管。我们首次开发了纤维化疾病模型，可用于研究皮肤和内脏纤维化的发展和后果。通过了解这些模型，很可能会出现更好的评估和治疗纤维化的方法。

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.