24 results for "Per Westermark"

Isolated atrial amyloidosis from atrial natriuretic peptide – a common but overlooked cardiac condition

Per Westermark; Gerhard Wikström; Per Eldhagen

Upsala Journal of Medical Sciences, Vol. 131 · 2026 · English · DOAJ Scopus

Interest in the misfolding and aggregation of human proteins into amyloid fibrils has increased dramatically in recent years. The systemic amyloidoses constitute a group of serious disorders that often affect the heart. Each systemic amyloid type is derived from one of several plasma proteins, most…

Fibrils from designed non-amyloid-related synthetic peptides induce AA-amyloidosis during inflammation in an animal model.

Per Westermark; Katarzyna Lundmark; Gunilla T Westermark

PLoS ONE, Vol. 4 No. 6 · 2009 · English · DOAJ Scopus

BACKGROUND:Mouse AA-amyloidosis is a transmissible disease by a prion-like mechanism where amyloid fibrils act by seeding. Synthetic peptides with no amyloid relationship can assemble into amyloid-like fibrils and these may have seeding capacity for amyloid proteins. PRINCIPAL FINDINGS:Several…

Also available at: PLOS

Regionalization of cell types in silk glands of Larinioides sclopetarius suggest that spider silk fibers are complex layered structures

Sumalata Sonavane; Per Westermark; Anna Rising et al.

Scientific Reports, Vol. 13 No. 1 · 2023 · English · DOAJ Scopus

Abstract In order to produce artificial silk fibers with properties that match the native spider silk we likely need to closely mimic the spinning process as well as fiber architecture and composition. To increase our understanding of the structure and function of the different silk glands of the…

Germ line origin and somatic mutations determine the target tissues in systemic AL-amyloidosis.

Stina Enqvist; Knut Sletten; Fred J Stevens et al.

PLoS ONE, Vol. 2 No. 10 · 2007 · English · DOAJ Scopus

BACKGROUND: Amyloid is insoluble aggregated proteins deposited in the extra cellular space. About 25 different proteins are known to form amyloid in vivo and are associated with severe diseases such as Alzheimer's disease, prion diseases and type-2 diabetes. Light chain (AL) -amyloidosis is unique…

Also available at: PLOS

Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition.

Sandra Arvidsson; Björn Pilebro; Per Westermark et al.

PLoS ONE, Vol. 10 No. 11 · 2015 · English · DOAJ Scopus

Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients -Type A…

Also available at: PLOS

Cardiac phenotype in hereditary transthyretin amyloidosis: correlations between fibril types and 99mTc-DPD uptake

Viktor Löfbacka; Jonas Wixner; Per Westermark et al.

Scientific Reports, Vol. 16 No. 1 · 2026 · English · DOAJ Scopus

Abstract Variant transthyretin amyloidosis is a systemic disease. In Sweden, the Val30Met variant is the most prevalent. Val30Met presents in two phenotypes: an early-onset form dominated by polyneuropathy and a late-onset form frequently accompanied by cardiomyopathy. These phenotypes are…

Tissue-based diagnosis of systemic amyloidosis: Experience of the informal diagnostic center at Uppsala University Hospital

Justina Damjanovic Vesterlund; Elisabet Ihse; Ulrika Thelander et al.

Upsala Journal of Medical Sciences, Vol. 127 · 2022 · English · DOAJ Scopus

Diagnosis of systemic amyloidosis is a clinical challenge and usually relies on a tissue biopsy. We have developed diagnostic methods based on the presence of amyloid deposits in abdominal subcutaneous fat tissue. This tissue is also used to determine the biochemical type of amyloidosis, performed…

The question of strains in AA amyloidosis

Gunilla T. Westermark; Ebba Nyström; Sofie Nyström et al.

Scientific Reports, Vol. 15 No. 1 · 2025 · English · DOAJ Scopus

…used to seed mouse AA amyloid in groups of inflamed NMRI mice (n = 9–10 per group). All but two mice showed amyloid deposits in the spleen induced by the human seeds. Amyloid binding ligand analysis was applied on the splenic amyloid deposits and revealed no clear significant difference between…

Amyloid nomenclature 2022: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee

Joel N. Buxbaum; Angela A. Dispenzieri; David S Eisenberg et al.

Amyloid · 2022 · English · OpenAlex Scopus

The Nomenclature Committee of the International Society of Amyloidosis met at the XVIII International Symposium on Amyloidosis in September and virtually in October 2022 with discussions resulting in this upgraded nomenclature recommendation. The nomenclature principles remain unchanged but there…

Post mortem findings and their relation to AA amyloidosis in free-ranging Herring gulls (Larus argentatus).

Désirée S Jansson; Caroline Bröjer; Aleksija Neimanis et al.

PLoS ONE, Vol. 13 No. 3 · 2018 · English · DOAJ Scopus

Since the late 1990s, high mortality and declining populations have been reported among sea birds including Herring gulls (Larus argentatus) from the Baltic Sea area in Northern Europe. Repeated BoNT type C/D botulism outbreaks have occurred, but it remains unclear whether this is the sole and…

Also available at: PLOS

AA amyloid in human food chain is a possible biohazard

Anna Rising; Paola Gherardi; Gefei Chen et al.

Scientific Reports, Vol. 11 No. 1 · 2021 · English · DOAJ Scopus

Abstract AA amyloidosis can be transmitted experimentally in several mammalian and avian species as well as spontaneously between captive animals, even by oral intake of amyloid seeds. Amyloid seeding can cross species boundaries, and fibrils of one kind of amyloid protein may also seed other…

Amyloid fibril composition within hereditary Val30Met (p. Val50Met) transthyretin amyloidosis families.

Ole Bernt Suhr; Jonas Wixner; Intissar Anan et al.

PLoS ONE, Vol. 14 No. 2 · 2019 · English · DOAJ Scopus

BACKGROUND:The amyloid fibril in hereditary transthyretin (TTR) Val30Met (pVal50Met) amyloid (ATTR Val30Met) amyloidosis is composed of either a mixture of full-length and TTR fragments (Type A) or of only full-length TTR (Type B). The type of amyloid fibril exerts an impact on the phenotype of the…

Also available at: PLOS

Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

Merrill Douglas Benson; Joel N. Buxbaum; David S Eisenberg et al.

Amyloid · 2020 · English · OpenAlex Scopus

The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September 2020 due to the ongoing COVID-19 pandemic instead of a planned meeting in Tarragona in March. In addition to…

Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis

Matthias Schmidt; Sebastian Wiese; Volkan Adak et al.

Nature Communications, Vol. 10 No. 1 · 2019 · English · DOAJ Scopus

Systemic amyloidosis of the ATTR is one of the most abundant forms of systemic amyloidosis and caused by misfolding of the circulating blood protein transthyretin (TTR). Here the authors present the cryo-EM structure of patient-derived Val30Met ATTR amyloid fibrils which reveals that the…

A father and his son with systemic AL amyloidosis

Stina Enqvist; Ulf-Henrik Mellqvist; Johan Mölne et al.

Haematologica, Vol. 94 No. 3 · 2009 · English · DOAJ Scopus