Conferences - Seminars

13SEP
2017
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  Wednesday 13 September 2017 12:15 - 13:15 SV1717

BMI Seminar // The role of proteolytic processing of the prion protein in neurodegenerative diseases

By Markus Glatzel Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Germany


Neurodegenerative diseases such as Alzheimer’s disease or prion diseases are untreatable debilitating diseases occurring mainly in the elderly.
For these diseases, molecular mechanisms of neurodegeneration are only partially understood. Aberrant proteolytic processing of neuronally expressed proteins leading to disturbed neuronal protein homeostasis represents one mechanism leading to neurodegeneration.
The membrane-anchored cellular prion protein plays detrimental roles in neurodegeneration by its ability to misfold into a pathogenic isoform (in prion diseases) and by acting as a neuronal receptor for toxic protein oligomers (e.g., in Alzheimer's disease). The mature form of the prion protein is processed by a variety of proteolytic cleavage events including shedding by the metalloprotease ADAM10.
Using cellular models and genetically modified mice, we study the impact of proteolytic processing of the cellular form of the prion protein on its physiological functions and its influence on neurodegenerative diseases.
Here, we report our most recent findings on the physiological and pathological relevance of these processing step and present an update on our experimental strategy of exploiting these cleavage events as a therapeutic option in neurodegenerative diseases.
 

Organization SV BMI Host : F. Hummel

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