Dementia in a dish
iPS-technology offers new platform to investigate frontotemporal dementia
Frontotemporal dementia (FTD) is a neurodegenerative disorder that causes neurons in the frontal and temporal lobes to die. In Germany alone, approximately 33,000 persons are affected by this disease. The exact mechanisms for the neurodegeneration are so far unknown. Therefore, there is no therapy that could cure or arrest FTD. Scientists of the Medical Faculty of the University of Münster and of the Max Planck Institute for Molecular Biomedicine have now used induced pluripotent stem cells (iPS cells) to develop a disease model for FTD in a petridish. With this technique, they succeeded to identify a gene that plays a protective role in affected neurons (Stem Cell Reports, epub July 2, 2015). Moreover, the researchers could show that the FTD-iPS cells could be used for drug screenings to develop a therapy for FTD.
Neurons differentiated from iPS cells from a healthy individual (left) and from a patient with with a FTD-associated tau mutation (right). Green marks neurons. The red colour shows a abnormal tau protein. The yellow/orange colour results from the overlay of both markers. Blue shows cell nuclei.
Tau protein aggregation in a FTD-patient:
The so called substantia nigra, a brain region that is important for planning and starting of movements, shows massive aggregations of abnormal tau proteins (brown staining).