CORDIS Project
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This project investigates the ribosome-associated quality control system, which is essential for degrading faulty proteins linked to neurodegeneration. By studying the mechanisms of this process in human cells, it aims to uncover how mutations affect neuronal function and contribute to diseases like Huntington's.
Ribosome-associated quality control (RQC) is crucial for degrading truncated nascent proteins produced on aberrant mRNAs.
This is done by elongation of the nascent chain on the large ribosomal subunit in the absence of mRNA and the small ribosomal subunit (CAT tailing) and by marking the nascent chain for degradation.
Mutations in RQC components cause neurodegeneration both in animal models and human patients.
Moreover, RQC insufficiency and subsequent protein aggregation critically contribute t…
Masarykova univerzita
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