source: University College London summary/abstract:
Amyloidosis is a rare disease caused by abnormal deposition and accumulation of proteins in the tissues of the body. Amyloid deposits are primarily made up of protein fibres known as amyloid fibrils. These amyloid fibrils are formed when normally soluble body proteins misfold and aggregate (clump together), and then remain in the tissues instead of safely going away.
The protein which misfolds and forms amyloid fibrils is known as a ‘precursor protein.’ About 30 different precursor proteins are known to form amyloid deposits in humans. Amyloidosis is usually a systemic disease. This means that many body organs and systems may be affected. Amyloidosis may also be ‘local’ or ‘localised’ which means that just a single organ or part of the body is affected. About 12% of all newly diagnosed amyloidosis patients at the National Amyloidosis Centre have localised amyloidosis.