Toxic Clumps

Within cells, the efficient disposal of dysfunctional and misfolded proteins is essential for the overall health and smooth running of the system. Indeed, impaired clearance of malformed proteins essentially clogs up the cell with waste and is an underlying pathology in several neurological and muscular degenerative diseases, including Alzheimer’s, Parkinson’s, ALS and frontotemporal dementia. Recent investigations into the mechanisms of protein disposal have identified a key enzyme – p97/VCP – essential for breaking down clumps of malformed proteins called aggresomes. The images show cells with normal (left) and reduced (right) levels of the enzyme, with the latter showing formation of the damaging aggresomes. Mutations to the p97/VCP enzyme have previously been linked to dementia and ALS. So, by picking apart this enzyme’s mode of action, scientists will glean not only an understanding about how pathology arises in these degenerative diseases but potentially how to tackle them.

Written by Ruth Williams

Ruth Williams

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Ruth is a freelance science journalist based in the US. She's a regular correspondent for The Scientist, and her work has appeared in The Lancet, Nature, Scientific American Mind, BBC Focus and elsewhere. Twitter @rooph

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• Image from work by Maria Körner and Paul Müller, and colleagues
• Biocenter, Chair of Biochemistry I, University of Würzburg, Würzburg, Germany
• Image originally published with a Creative Commons Attribution 4.0 International (CC BY 4.0)
• Research published in Nature Communications, May 2025