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Tag Archives: protein-folding disorders

July 2008 Br J Cardiol 2008;15:177–8

Amyloid heart disease – the continuing enigma

Simon Dubrey

Abstract

The amyloidoses form a collection of diseases that are part of the wider category of protein-folding disorders. Within this spectrum, proteins comprising both normal plasma constituents and mutations of such proteins, undergo a change in conformation. Such proteins become ‘insoluble’ and form highly ordered fibrillar aggregates. In amyloidosis, a process of self-assembly occurs in various tissues to form a beta-pleated sheet. While a comparatively rare condition, it is frequently not recognised or misdiagnosed. Patients presenting with advanced cardiac involvement have an extremely poor prognosis, with survival measureable in terms of onl

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