Protein Misfolding, ER-Stress and Protein Degradation - Development of a Systematic Pipeline for Individualised Therapy in Hereditary Disorders of the Pancreas and Liver
In the last few years it has been discovered that “Endoplasmic Reticulum Stress” is an important development mechanism for various diseases. This leads to a significant interference in the processing of proteins in body cells and this results in an accumulation of misfolded proteins. These then have to be degraded in order to avoid cell damage caused by permanently stored proteins. The mechanism has already been identified in several hereditary storage diseases, in liver disorders and types of inflammation of the pancreas (pancreatitis). It leads to inflammation, the destruction of tissue and to chronic organ damage. The clinical scientists from Greifswald and Rostock who are involved in the project, are experts for storage diseases and pancreas disorders and have now received a grant of 5 million euros from EU funds for a period of 4 years, as part of the state of Mecklenburg-Vorpommern’s Research Excellence Programme. During this period, they will not only examine the basic mechanisms of hereditary liver and pancreas disorders in further depth, but will also work simultaneously with partners carrying out fundamental research to develop new therapeutic substances, which either inhibit ER-Stress or repair the cell’s defective degradation of false proteins.
The project is coordinated by University Medicine Greifswald with partners from the University of Greifswald, the Albrecht-Kossel-Institute and the Institute of Chemistry at the University of Rostock, and the Leibniz Institute for Catalysis in Rostock.
Excerpt from the jury’s vote:
“The chosen approach of synthesising chaperones is seen as innovative. The Jury also perceives the chosen strategy of first examining illnesses with marginal patient relevance (small number of illnesses) and superior groundwork (Johanson-Blizzard Syndrome, Wilson’s Disease) to later apply the results to more complex illnesses, such as Parkinson’s and Alzheimer’s and to thus continue the research work, to be promising."