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Structure paper

TitleStructural and molecular homogeneity of ATTRv-T60A amyloid fibrils across patients and organs.
Journal, issue, pagesStructure, Vol. 33, Issue 12, Page 2013-22019.e4, Year 2025
Publish dateDec 4, 2025
AuthorsMaria Del Carmen Fernandez-Ramirez / Binh A Nguyen / Shumaila Afrin / Virender Singh / Bret Evers / John M Shelton / Christian Lopez Escobar / Parker Bassett / Lanie Wang / Maja Pękała / Yasmin Ahmed / Luis O Cabrera Hernandez / Rose Pedretti / Preeti Singh / Jacob Canepa / Aleksandra Wosztyl / Yang Li / David R Boyer / Qin Cao / Lorena Saelices /
PubMed AbstractTransthyretin amyloidosis is a systemic protein misfolding disorder with diverse clinical phenotypes, including cardiomyopathy, polyneuropathy, or a combination of both. While structural polymorphism ...Transthyretin amyloidosis is a systemic protein misfolding disorder with diverse clinical phenotypes, including cardiomyopathy, polyneuropathy, or a combination of both. While structural polymorphism of amyloid fibrils has been linked to disease heterogeneity in neurodegenerative disorders, its role in transthyretin amyloidosis remains unclear. Here, we used cryo-electron microscopy to analyze ex vivo fibrils extracted from the hearts of three patients carrying the T60A mutation, a variant associated with mixed cardiac and neuropathic symptoms. In one patient, we additionally examined fibrils from the thyroid, kidney, and liver. All fibrils across patients and tissues adopted a single morphology previously associated with cardiomyopathy. Complementary molecular analyses revealed high compositional homogeneity. Notably, we extracted fibrils from the liver, an organ considered fibril-free, with seeding capacity in vitro. These findings suggest structural homogeneity as a hallmark of cardiac and mixed phenotypes, and provide a mechanistic rationale for the transmission of amyloidosis following domino liver transplantation.
External linksStructure / PubMed:41106377 / PubMed Central
MethodsEM (helical sym.)
Resolution2.98 - 4.64 Å
Structure data

46479

9d21

EMDB-46479, PDB-9d21:
Cryo-EM structure of amyloid fibril extracted from heart of a variant ATTR T60A amyloidosis patient 1
Method: EM (helical sym.) / Resolution: 3.4 Å

46481

9d23

EMDB-46481, PDB-9d23:
Cryo-EM structure of amyloid fibril extracted from heart of a variant ATTR T60A amyloidosis patient 2
Method: EM (helical sym.) / Resolution: 3.18 Å

46482

9d24

EMDB-46482, PDB-9d24:
Cryo-EM structure of amyloid fibril extracted from thyroid of a variant ATTR T60A amyloidosis patient 3
Method: EM (helical sym.) / Resolution: 2.98 Å

46487

9d27

EMDB-46487, PDB-9d27:
Cryo-EM structure of amyloid fibril extracted from kidney of a variant ATTR T60A amyloidosis patient 3
Method: EM (helical sym.) / Resolution: 3.19 Å

46495

9d2g

EMDB-46495, PDB-9d2g:
Cryo-EM structure of amyloid fibril extracted from liver of a variant ATTR T60A amyloidosis patient 3
Method: EM (helical sym.) / Resolution: 3.1 Å

EMDB-46496: Cryo-EM structure of amyloid fibril extracted from heart of a variant ATTR T60A amyloidosis patient 3
Method: EM (helical sym.) / Resolution: 4.64 Å

Source
  • homo sapiens (human)
KeywordsPROTEIN FIBRIL / Transthyretin / Amyloidosis / ATTR / Cardiac / Thyroid / Kidney / liver

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