Decreased CCN3 in Systemic Sclerosis endothelial cells contributes to impaired angiogenesis

Systemic sclerosis (SSc) is a rare and severe connective tissue disease combining auto-immune and vasculopathy features, ultimately leading to organ fibrosis. Impaired angiogenesis is an often silent and life-threatening complication of the disease. We hypothesize that CCN3 (NOV), a member of the CCN family of extra-cellular matrix proteins, which is an antagonist of the pro-fibrotic protein CCN2 (CTGF) as well as a pro-angiogenic factor, is implicated in SSc pathophysiology.We performed skin biopsies on 26 SSc patients, both in fibrotic and non-fibrotic areas for 17 patients, and collected 18 healthy control skin specimens, for immunohistochemistry and cell culture.

Source: Journal of Investigative Dermatology - January 15, 2020 Category: Dermatology Authors: Pauline Henrot, Fran çois Moisan, Paôline Laurent, Pauline Manicki, Priscilla Kaulanjan-Checkmodine, Valérie Jolivel, Hamid Reza Rezvani, Vaianu Leroy, François Picard, Carine Boulon, Thierry Schaeverbeke, Julien Seneschal, Estibaliz Lazaro, Alain Taà Tags: Original Article Source Type: research