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TitleAmyloid fibril structures link CHCHD10 and CHCHD2 to neurodegeneration.
Journal, issue, pagesNat Commun, Vol. 16, Issue 1, Page 7121, Year 2025
Publish dateAug 2, 2025
AuthorsGuohua Lv / Nicole M Sayles / Yun Huang / Chiara Mancinelli / Kevin McAvoy / Neil A Shneider / Giovanni Manfredi / Hibiki Kawamata / David Eliezer /
PubMed AbstractMitochondrial 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 ...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 the CHCHD10 and CHCHD2 fibril structures share structural similarities and appear compatible with sequence variations in this region. In contrast, disease-associated mutations p.S59L in CHCHD10 and p.T61I in CHCHD2, situated within the ordered cores of these fibrils, cannot be accommodated by the wildtype structures and promote different protofilament folds and fibril structures. These results link CHCHD10 and CHCHD2 amyloid fibrils to neurodegeneration and further suggest that fibril formation by the WT proteins could also be involved in disease etiology.
External linksNat Commun / PubMed:40753073 / PubMed Central
MethodsEM (helical sym.)
Resolution2.03 - 3.15 Å
Structure data

45976

9cww

EMDB-45976, PDB-9cww:
Structure of D10-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 2.3 Å

71028

9oyo

EMDB-71028, PDB-9oyo:
Structure of R15L D10-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 3.15 Å

71031

9oyq

EMDB-71031, PDB-9oyq:
Structure of D10-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 2.7 Å

71032

9oyr

EMDB-71032, PDB-9oyr:
Structure of D2-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 2.03 Å

71033

9oys

EMDB-71033, PDB-9oys:
Structure of S59L D10-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 3.06 Å

71034

9oyt

EMDB-71034, PDB-9oyt:
Structure of T61I D2-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 3.1 Å

71037

9oyw

EMDB-71037, PDB-9oyw:
Structure of D10-NT amyloid fibrils
Method: EM (helical sym.) / Resolution: 2.63 Å

Source
  • homo sapiens (human)
KeywordsPROTEIN FIBRIL / CHCHD10 / Amyloid Fibril / R15L / CHCHD2 / S59L of CHCHD10 / T61I CHCHD2

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