[Mechanism of beta(2)-microglobulin-related amyloid fibril formation in CKD].
Dialysis-related amyloidosis is a serious complication in patients on long-term dialysis. Amyloid fibrils, that major component is beta(2)-microglobulin (beta(2)-m) , deposit mainly in osteoarticular joint tissues and cause various complications such as carpal tunnel syndrome, destructive spondyloarthropathy, bone cysts, and various organ dysfunction. The retention of beta(2)-m in chronic kidney disease (CKD) induces the amyloid fibril formation according to a nucleation-dependent polymerization model. Specific molecules such as glycosaminoglycans and proteoglycans that affect the conformation and stability of beta(2)-m and amyloid fibrils are considered to have significant effects on the formation and deposition of amyloid fibrils.
Yamamoto
Suguru
S
Niigata University Graduate School of Medicine and Dental Scineces, Division of Clinical Nephrology and Rheumatology, Japan.
Kazama
Junichiro James
JJ
D000682:Amyloid / Q000096:biosynthesis*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000378:metabolism*; D001613:beta 2-Microglobulin / Q000378:metabolism*
D000682:Amyloid; D001613:beta 2-Microglobulin