Elsevier

Laboratory Investigation

Volume 96, Issue 2, February 2016, Pages 151-155
Laboratory Investigation

Pathobiology in Focus
Canonical Wnt signaling in systemic sclerosis

https://doi.org/10.1038/labinvest.2015.154Get rights and content
Under an Elsevier user license
open archive

Abstract

Fibrosing disorders are characterized by abundant accumulation of extracellular matrix proteins such as collagen in a variety of organs, which results in structural changes and dysfunction of the affected organ. Thus fibrotic diseases are characterized by a high morbidity and mortality and also lead to major socioeconomic costs. Systemic sclerosis (SSc) is a prototypic multi-systemic fibrosing disease, which affects the skin and a variety of internal organs, including the lungs, heart and gastrointestinal tract. Targeted antifibrotic therapies are not yet available for clinical use in SSc. In recent years, canonical Wnt signaling has been profoundly characterized as an important mediator of sustained fibroblast activation in fibrotic diseases. In the present review, we will summarize current research on the canonical Wnt signaling pathway in SSc and discuss translational implications and potential limitations of prolonged Wnt inhibition.

Cited by (0)

This review comprehensively describes the pivotal role of Wnt/β-catenin in systemic sclerosis. The emerging story of this fibrotic disease which affects not only the skin but the gastrointestinal system, lungs and heart, is the relationship of TGF-b with increased Wnt/β-catenin signaling and the amelioration of fibrosis with Dkk-1. Both translational implications and the limitations of prolonged Wnt/β-catenin inhibition are discussed.