Influence of metabolism on fibrosis in SSc

Concept

Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown cause, which leads to disability and may cause premature death. SSc is characterized by massive accumulation of extracellular matrix proteins (=fibrosis) in skin and internal organs, such as lungs, with permanent loss of organ function.

Metabolic alterations have been recently recognized as an important pathogenic process underlying fibrosis. Decreasing/reversing fibrosis in patients with SSc can improve the prognosis of this devastating disease.

Facts and Figures

Project Lead
B Burja
University Medical Centre Ljubljana
FOREUM research grant: € 150.000
Duration 3 years

Meet the Team

Project Lead

B Burja
University Medical Centre Ljubljana
M Frank-Bertoncelj
University of Zurich
K Lakota
University Medical Centre Ljubljana
O Distler
University of Zurich
M Tomšič
University Medical Centre Ljubljana

Objectives

We aim at exploring the crosstalk between metabolic and fibrotic pathways in SSc fibroblasts to uncover new anti-fibrotic  treatment strategies. We will explore dysregulation of metabolic pathways in SSc fibroblasts and determine, whether metabolic substrates, such as alpha-ketoglutarate (αKG) influence the pro-fibrotic activities of skin and lung fibroblasts. Targeting metabolic pathways might reverse fibrotic activation and halt fibrosis in SSc with direct implications for drug discovery in SSc.

Patient Voice

Patients with SSc will be involved in the preparation of informed consent forms and communicating our research findings to public. Patients will actively participate in the development of research projects. We will promote the EULAR’s initiative ‘Patients - research partners’. This will establish long-term partnerships between rheumatologists, researchers and patients in Slovenia.

Project Map