Article

Amyloid fibril structures link CHCHD10 and CHCHD2 to neurodegeneration

2024-07-22

Abstract excerpt

CHCHD10 is mutated in rare cases of FTD and ALS and aggregates in mouse models of disease. Here we show that the disordered N-terminal domain of CHCHD10 forms amyloid fibrils and report their cryoEM structure. Disease-associated mutations cannot be accommodated by the WT fibril structure, while sequence differences between CHCHD10 and CHCHD2 are tolerated, explaining the co-aggregation of the two proteins and link...

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Literature Corpus work: 9f3c1145-42fa-5916-bed1-e9b9eeb1e50a
DOI: 10.1101/2024.07.18.604174

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