Hereditary Fibrinogen Aα-Chain Amyloidosis | oneAMYLOIDOSISvoice
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Hereditary Fibrinogen Aα-Chain Amyloidosis

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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 being safely cleared away. About 30 different proteins are known to form amyloid deposits in humans. 

These amyloid forming 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.

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