6 results for "Per Westermark"
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Fibrils from Designed Non-Amyloid-Related Synthetic Peptides Induce AA-Amyloidosis during Inflammation in an Animal Model
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…
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Germ Line Origin and Somatic Mutations Determine the Target Tissues in Systemic AL-Amyloidosis
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 Alzheimeŕs disease, prion diseases and type-2 diabetes. Light chain (AL) -amyloidosis is unique…
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Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition
Purpose: 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…
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Post mortem findings and their relation to AA amyloidosis in free-ranging Herring gulls ( Larus argentatus )
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…
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Amyloid fibril composition within hereditary Val30Met ( p . Val50Met ) transthyretin amyloidosis families
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…
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Carbonic Anhydrase Generates CO 2 and H + That Drive Spider Silk Formation Via Opposite Effects on the Terminal Domains
Mapping the conditions of spider silk proteins along the silk gland, and combining with molecular studies, reveals a pH controlled switch between lock and trigger forms, providing insights into spider silk formation. Spider silk fibers are produced from soluble proteins (spidroins) under ambient…