Article
Amyloid fibril structures link CHCHD10 and CHCHD2 to neurodegeneration.
Nature communications - 2 Aug 2025
Lv Guohua, Sayles Nicole M, Huang Yun, Mancinelli Chiara, McAvoy Kevin, Shneider Neil A, Manfredi Giovanni, Kawamata Hibiki, Eliezer David
Abstract excerpt
Mitochondrial proteins CHCHD10 and CHCHD2 are mutated in rare cases of heritable FTD, ALS and PD and aggregate in tissues affected by these diseases. Here, we show that both proteins form amyloid fibrils and report cryo-EM structures of fibrils formed from their disordered N-terminal domains. The ordered cores of these fibrils are comprised of a region highly conserved between the two proteins, and a subset of...
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