Although the LCSB has long been known for its research in the field of Parkinson’s disease, an increasing number of scientists from the LCSB also work on other neurodegenerative diseases, especially those affecting children. These are often rare diseases that are usually hard to diagnose and that are neglected by pharma companies due to their lack of profitability.
In 2017, the Luxembourg Centre for Systems Biomedicine (LCSB) launched a research project on Zellweger Syndrome, one of several thousand rare childhood diseases, and part of the leukostrophy disease group.
Zellweger syndrome is caused by mutations in a group of genes coding for peroxins, which are proteins required for the normal assembly of peroxisomes. The major role of these peroxisomes is the breakdown of very long chain fatty acids. In Zellweger syndrome, these peroxisomes are defective, leading to the accumulation of these long molecules in the cells. As these molecules keep accumulating, they turn into toxic waste thereby affecting the cells of multiple organs in the human body, including the brain and the liver. Currently, there is no cure, nor treatment for this rare disease that almost always leads to the premature death of the affected children.
The project, which is one of several LCSB studies on childhood diseases, was initiated by the parents of several affected kids. Unable to help their children, these parents reached out to the LCSB and asked for help.
The generous financial support of several private donors (including affected family members), the Fondation André et Henriette Losch, the companies Evertz Europe AG and Evertz GmbH & Co. KG, and the Rotary Club Bascharage-Kordall has made it possible for the LCSB to hire an experienced researcher whose time will be completely devoted to the study of Zellweger syndrome: Scientist Zlatan Hodzic moved from San Diego to Belval in November 2017 to work on Zellweger Syndrome under the guidance of Dr Carole Linster with the goal to carry out drug discovery screens on human cells affected by the disease.
For more information on how you can help support this project please contact philippe.lamesch@uni.lu
Most recent donation towards Zellweger research: Luxembourg’s Rotary Club Bascharage-Kordall presents 5,000 EUR Cheque to LCSB’s Principal Investigator Dr. Carole Linster.