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 aggregate (clump together) and then remain in the tissues instead of safely going away. About 30 different proteins are known to form amyloid deposits in humans.
These amyloid forming (‘amyloidogenic’) proteins are known as ‘precursor proteins.’ Amyloid deposits cause disease by gradually accumulating within organs and thereby disrupting the structure and damaging the function of the affected tissues.
Different types of amyloidosis are named according to the precursor proteins which form the amyloid fibrils. All have the initial ‘A’ denoting amyloidosis and letter(s) identifying the particular precursor protein which forms amyloid fibrils within the amyloid deposits.
In ATTR amyloidosis, a blood protein called transthyretin (TTR) is the amyloid precursor protein that forms the amyloid deposits. Wild-type ATTR amyloidosis, formerly known as senile systemic amyloidosis, is one of three distinct, different types of ATTR amyloidosis. read more