X-linked inhibitor of apoptosis protein (XIAP) inhibition in systemic sclerosis (SSc).

Christina BergmannLudwig HallenbergerSara Chenguiti FakhouriBenita MerlevedeAmelie BrandtClara Dees Honglin ZhuAriella ZehenderXiang ZhouAnnemarie SchwabChih-Wei ChenAndrea Hermina GyörfiAlexandru Emil MateiDebomita ChakrabortyThuong Trinh-MinhSimon RauberRoland CorasAline BozecAlexander KreuterMirjana ZiemerLarissa Valor-Méndez Jörg H W Distler

Published in: Annals of the rheumatic diseases (2021)

Our data characterise XIAP as a novel link between two core pathways of fibrosis. XIAP is overexpressed in SSc and cGvHD in a TGF-β/SMAD3-dependent manner and in turn amplifies the profibrotic effects of WNT/β-catenin signalling on fibroblasts via transducin-like enhancer of split 3. Targeted inactivation of XIAP inhibits the aberrant activation of fibroblasts in murine models of SSc.

Keyphrases

systemic sclerosis
interstitial lung disease
epithelial mesenchymal transition
transforming growth factor
cell proliferation
oxidative stress
stem cells
binding protein
endoplasmic reticulum stress
electronic health record
transcription factor
cancer therapy
big data
rheumatoid arthritis
amino acid
pi k akt