Protein-misfolding diseases: Getting out of shape - Nature

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Two greatly debilitating illnesses — Alzheimer's disease, which is largely associated with ageing, and variant Creutzfeldt–Jakob disease (vCJD), which is linked with the recent bovine spongiform encephalopathy (BSE) epidemic in Britain — have had a huge impact on public consciousness. Despite their very different origins, these diseases are closely related to each other and also to the 20 or so other 'amyloidoses' — so called because they involve the aberrant deposition of proteins in the form of amyloid fibrils or plaques. Other diseases in this group, which are less publicized but no less devastating to those affected, are Parkinson's disease, type II (late-onset) diabetes and rare conditions such as familial insomnia.

Amyloidoses thus include some of the most feared and costly diseases in the Western world. Alzheimer's may very soon be the most prevalent and socially disruptive illness in the ageing populations of all developed countries. It is remarkable, therefore, that most of these diseases were virtually unknown until relatively recently. Indeed, the first detailed description of an amyloid pathology was made less than 100 years ago, when Alzheimer identified the form of dementia that bears his name. As with many amyloidoses, Alzheimer's disease is usually sporadic, although there are less common hereditary forms that can afflict relatively young people — Alzheimer's most famous patient was in her early 50s.

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