CCN proteins as potential actionable targets in scleroderma.

Abstract:

:Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease combining inflammatory, vasculopathic and fibrotic manifestations. Skin features, which give their name to the disease and are considered as diagnostic as well as prognostic markers, have not been thoroughly investigated in terms of therapeutic targets. CCN proteins (CYR61/CCN1, CTGF/CCN2, NOV/CCN3 and WISP1-2-3 as CCN4-5-6) are a family of secreted matricellular proteins implicated in major cellular processes such as cell growth, migration, differentiation. They have already been implicated in key pathophysiological processes of SSc, namely fibrosis, vasculopathy and inflammation. In this review, we discuss the possible implication of CCN proteins in SSc pathogenesis, with a special focus on skin features, and identify the potential actionable CCN targets.

journal_name

Exp Dermatol

journal_title

Experimental dermatology

authors

Henrot P,Truchetet ME,Fisher G,Taïeb A,Cario M

doi

10.1111/exd.13806

subject

Has Abstract

pub_date

2019-01-01 00:00:00

pages

11-18

issue

1

eissn

0906-6705

issn

1600-0625

journal_volume

28

pub_type

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