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Amyloid fibril composition type is consistent over time in patients with Val30Met (p. Val50Met) transthyretin amyloidosis
Umeå University, Faculty of Medicine, Wallenberg Centre for Molecular Medicine at Umeå University (WCMM). Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Section of Medicine.ORCID iD: 0000-0003-2874-7643
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Section of Medicine.ORCID iD: 0000-0002-1175-2369
Department of Medicine, Piteå Hospital, Piteå, Sweden.
Department of Medicine, Piteå Hospital, Piteå, Sweden.
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2022 (English)In: PLOS ONE, E-ISSN 1932-6203, Vol. 17, no 3, article id e0266092Article in journal (Refereed) Published
Abstract [en]

Background: We have previously shown that transthyretin (TTR) amyloidosis patients have amyloid fibrils of either of two compositions; type A fibrils consisting of large amounts of C-terminal TTR fragments in addition to full-length TTR, or type B fibrils consisting of only full-length TTR. Since type A fibrils are associated with an older age in ATTRVal30Met (p.Val50Met) amyloidosis patients, it has been discussed if the TTR fragments are derived from degradation of the amyloid deposits as the patients are aging. The present study aimed to investigate if the fibril composition type changes over time, especially if type B fibrils can shift to type A fibrils as the disease progresses.

Material and methods: Abdominal adipose tissue biopsies from 29 Swedish ATTRVal30Met amyloidosis patients were investigated. The fibril type in the patients initial biopsy taken for diagnostic purposes was compared to a biopsy taken several years later (ranging between 2 and 13 years). The fibril composition type was determined by western blot.

Results: All 29 patients had the same fibril composition type in both the initial and the follow-up biopsy (8 type A and 21 type B). Even patients with a disease duration of more than 12 years and an age over 75 years at the time of the follow-up biopsy had type B fibrils in both biopsies.

Discussion: The result clearly shows that the amyloid fibril composition containing large amounts of C-terminal fragments (fibril type A) is a consequence of other factors than a slow degradation process occurring over time.

Place, publisher, year, edition, pages
Public Library of Science , 2022. Vol. 17, no 3, article id e0266092
National Category
Cardiac and Cardiovascular Systems
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URN: urn:nbn:se:umu:diva-193805DOI: 10.1371/journal.pone.0266092ISI: 000799828800066PubMedID: 35358243Scopus ID: 2-s2.0-85127435798OAI: oai:DiVA.org:umu-193805DiVA, id: diva2:1656531
Funder
Erik, Karin och Gösta Selanders FoundationAvailable from: 2022-05-06 Created: 2022-05-06 Last updated: 2023-09-05Bibliographically approved

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Anan, IntissarSuhr, Ole B.Pilebro, BjörnWixner, Jonas

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