Exposure of a cryptic Hsp70 binding site determines the cytotoxicity of the ALS-associated SOD1-mutant A4V.

doi:10.1093/protein/gzaa008

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Exposure of a cryptic Hsp70 binding site determines the cytotoxicity of the ALS-associated SOD1-mutant A4V.

Protein engineering, design & selection : PEDS - 31 Dec 2019

Claes Filip, Rudyak Stanislav, Laird Angela S, Louros Nikolaos, Beerten Jacinte, Debulpaep Maja, Michiels Emiel, van der Kant Rob, Van Durme Joost, De Baets Greet, Houben Bert, Ramakers Meine, Yuan Kristy, Gwee Serene S L, Hernandez Sara, Broersen Kerensa, Oliveberg Mikael, Moahamed Barbara, Kirstein Janine, Robberecht Wim, Rousseau Frederic, Schymkowitz Joost

Abstract excerpt

The accumulation of toxic protein aggregates is thought to play a key role in a range of degenerative pathologies, but it remains unclear why aggregation of polypeptides into non-native assemblies is toxic and why cellular clearance pathways offer ineffective protection. We here study the A4V mutant of SOD1, which forms toxic aggregates in motor neurons of patients with familial amyotrophic lateral sclerosis...

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PMID: 32399571
DOI: 10.1093/protein/gzaa008

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Source receipt: pubmed, retrieved 7 Sept 2026.

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