Opening with a view of the heart, we reveal several amyloid deposits infiltrating the muscle tissue.
Then we zoom down to the heart muscle cells and see misfolded light chains aggregating into insoluble amyloid fibrils. We fade, over time, from the early onset of light-chain infiltration to late-stage fibril aggregation, where the buildup of amyloid deposits around and in between the cardiomyocytes has made the muscle tissue stiff and less flexible, impairing the heart’s ability to relax and fill with blood.
Amyloid light chain (AL) amyloidosis is the most common form of systemic amyloidosis and is caused by the misfolding of immunoglobulin light chains, which are produced by abnormal plasma cells in the bone marrow.
Deposits can be widespread, affecting multiple organs, or they can be more localized.