Amyloidosis refers to a complex group of clinical syndromes caused by deposition of misfolded proteins with a beta pleated sheet confirmation resulting in organ damage in various tissues.
Currently, there are 36 different proteins recognized to have amylodogenic potential.
It is the extracellular deposition of amyloid proteins fibrils that lead to organ damage.
Clinical manifestations of amyloidosis are determined by the abnormal protein, its organ distribution, and whether the disorder is hereditary.
Therefore accurate determination of the amyloid proteins has become mandatory.
The most common recognizeD amyloid type is systemic AL amyloidosis due to deposition of immunoglobulin light chains produced by a bone marrow based monotypic plasma cell population.
Wild type ATTR amyloidosis is probably the most common type of systemic amyloidosis, which results from deposition of wild type ATTR and typically involves the heart.
Mass spectrometry is the gold standard for typing amyloid fibrils.