1996 …2023

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Huntington’s disease (HD) is hallmarked by the accumulation and aggregation of mutant huntingtin protein (mHTT) fragments. Our goal is to improve recognition and degradation of mHTT by the ubiquitin-proteasome system.

An increasing number of neurodegenerative disorders is characterized by the accumulation and aggregation of particular proteins. Here, aggregates are often formed by proteolytic fragments and not by full-length proteins, as is the case with Huntington's and Alzheimer's disease. This suggests that they share a common mechanism in the cellular inability to remove certain peptides which start forming toxic oligomeric structures that may kill the cell unless stored into protective aggresomes. Peptidases may play a crucial role in the cellular attempt to degrade the monomeric fragments once released from the original proteins, and may even play in concert with heat shock proteins to dismantle oligomeric structures into fragments that can subsequently be degraded.



Ubiquitin-Proteasome System, Huntington's Disease, head core facility Cellular Imaging


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