| [ Summary ] |
Amyloidosis is a group of precursor protein misfolding diseases characterized by the pathological deposition in extracellular spaces with insoluble fibrils. Dialysis-related amyloidosis (DRA) is a common, serious complication in patients receiving long-term dialysis. DRA is regarded as one of the systemic forms of amyloidosis because of the deposits made in the chorda, bone, synovia, heart, or skin. The most common manifestations are carpal tunnel syndrome, bone cysts, and destructive spondyloarthropathy. Since Gejyo displayed, in 1985, that beta2-microglobulin (beta2-m) was a major structural component of DRA fibrils, many investigators have reported studies on DRA. Although the structural stability of beta2-m may help in understanding the molecular bases of DRA fibril formation, the detailed mechanism is still obscure. In this paper, we described an outline of the study for DRA, epidemiology, risk factors, commonality compared with other forms of amyloidosis and specificity. In addition, we also mentioned a characteristic of beta2-m, it's role, in the kinetics of the body. |