(July 27, 2015) Scientists of the Comprehensive Pneumology
Center (CPC) at the Helmholtz Zentrum München have identified a new mechanism
which contributes to the development of idiopathic pulmonary fibrosis (IPF).
They showed that the pathological changes of lung tissue are accompanied by an
increase in protein turnover by the central protein degradation machinery of
the cell – the proteasome. Their study has now been published in the ‘American
Journal of Respiratory and Critical Care Medicine’.
Idiopathic pulmonary fibrosis is a very aggressive form of
pulmonary fibrosis and has a particularly poor prognosis. This fatal disease,
for which so far no causal therapies exist, is characterized by a massive
deposition of connective and scar tissue in the lung, which leads to a
progressive loss of lung function and ultimately death. Connective tissue is
mainly produced by myofibroblasts. The research group led by PD Dr. Silke
Meiners of the Institute of Lung Biology and the CPC showed now for the first time
that the activation of these myofibroblasts depends on increased protein
turnover by the 26S proteasome*.