CCN3: a novel antifibrotic treatment?

硬皮病等纤维性疾病没有治疗方法。这些疾病的特点是广泛的疤痕组织沉积。由于周围的疤痕组织，疤痕内的细胞(成纤维细胞)会受到增强的张力。它们本身也是不正常的，因为它们对疤痕组织施加了过度的张力。人们认为，这种过度的尺寸是导致疤痕保持的原因。一种名为CCN2的蛋白质在疤痕组织中特异表达，促进了这种张力。在疤痕组织中没有发现一种相关的蛋白质CCN3，但通常会抑制CCN2。我们想要测试CCN3(以及CCN3衍生的多肽)是否可以阻止硬皮病纤维化。此外，我们希望测试监测患者的CCN2/CCN3比率是否可以用于监测疾病进展在资助期结束时，我们将设计并启动我们的多肽临床试验，看看我们是否可以在临床上阻止纤维化。

Fibrotic diseases such as scleroderma have no therapy. These diseases are characterized by extensive scar tissuedeposition. The cells (fibroblasts) within the scar are subject to enhanced tension due to the surrounding scar tissue. Theyalso themselves are abnormal in that they exert excessive tension on the scar tissue. It is believed that this excessivetension is responsible for the maintenance of the scar. A protein called CCN2 is expressed specifically in scar tissue andpromotes this tension. A related protein CCN3 is not found in scar tissue, but normally acts to inhibit CCN2. We want to testwhether CCN3 (and peptides derived from CCN3) can block scleroderma fibrosis. Moreover, we want to test if monitoringthe CCN2:CCN3 ratio in patients can be used to monitor disease progression At the end of the funding period, we will designandinitiate clinical trials with our peptides to see if we can block fibrosis clinically.