The age related amyloids: a growing family of unique biochemical substances

摘要

The age related amyloids represent an extremely varied group of pathological entities. Some forms of amyloid, such as aortic and choroid plexus, are present in virtually all elderly people. On the basis of currently available information, all major forms of senile amy-loidosis seem to be derived from normally synthesised proteins. TTR seems to have the most distinctive characteristics of all the amyloid fibril proteins of producing a generally benign amyloidosis in its native form (SSA) and a malignant, lethal amyloidosis in its mutant form (FAP). Many of the localised age related amyloids do not appear to cause clinical disease. However, although the complex mechanisms are not completely understood, the P protein and LAPP derived amyloids are generally considered to have significant links to their respective associated clinical diseases (Alzheimer's disease and type 2 diabetes mellitus). Although the precursor protein of the intimal type of senile aortic amyloid is unknown, its relation to atherosclerosis deserves further study. Irrespective of how the debate regarding the pathological significance of the age related amyloids evolves, further characterisation of their fibril protein structures promises to advance our understanding of the entire complex and puzzling family of amyloidoses.