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TitleAmyloid fibril polymorphism in the heart and liver of a patient with polyneuropathic ATTRv-V122Δ amyloidosis.
Journal, issue, pagesCommun Biol, Year 2026
Publish dateApr 1, 2026
AuthorsYasmin Ahmed / Binh An Nguyen / Candace Kelly / Shumaila Afrin / Virender Singh / Bret M Evers / John M Shelton / Christian Lopez Escobar / Preeti Singh / Rose Pedretti / Lanie Wang / Parker Bassett / Maria Del Carmen Fernandez-Ramirez / Maja Pekala / Andrew Lemoff / Barbara Kluve-Beckerman / Lorena Saelices /
PubMed AbstractATTR amyloidosis is a phenotypically heterogeneous disease characterized by the pathological deposition of transthyretin in the form of amyloid fibrils into various organs. ATTR amyloidosis may ...ATTR amyloidosis is a phenotypically heterogeneous disease characterized by the pathological deposition of transthyretin in the form of amyloid fibrils into various organs. ATTR amyloidosis may result from mutations in variant (ATTRv) amyloidosis, or aging in wild-type (ATTRwt) amyloidosis. ATTRwt generally manifests as cardiomyopathy, whereas ATTRv may present as polyneuropathy, cardiomyopathy, or mixed, in combination with many other symptoms deriving from multisystem organ involvement. Over 220 different mutational variants of transthyretin have been identified, many of them being linked to specific disease symptoms. Yet, the role of these mutations in explaining differential disease manifestations remains unclear. Using cryo-electron microscopy, here we structurally characterized fibrils from the heart and the liver of an ATTRv patient carrying the V122∆ mutation, which is predominantly associated with polyneuropathy. Our results show that these fibrils are polymorphic, presenting as both single and double filaments. Our study alludes to a structural connection contributing to phenotypic variation in ATTR amyloidosis, as polymorphism in ATTR fibrils may manifest in patients with predominantly polyneuropathic phenotypes.
External linksCommun Biol / PubMed:41917523
MethodsEM (helical sym.)
Resolution2.9 Å
Structure data

74399

9zld

EMDB-74399, PDB-9zld:
Cryo-EM structure of hepatic amyloid fibril from a variant ATTRV122delta, single filament morphology
Method: EM (helical sym.) / Resolution: 2.9 Å

Source
  • homo sapiens (human)
KeywordsPROTEIN FIBRIL / ATTR / Systemic amyloidosis / V122delta / amyloid

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