Beta-2-microglobulin amyloid fibril (Abeta2M)

Beta-2-microglobulin (B2M) amyloid fibrils are pathological protein aggregates primarily composed of the B2M protein, which normally serves as the light chain of the Major Histocompatibility Complex (MHC) class I molecule [UniProt P61769]. In individuals with chronic kidney disease, particularly those on long-term hemodialysis, B2M accumulates in the blood due to impaired renal clearance, eventually misfolding and polymerizing into insoluble amyloid fibrils [StatPearls, 2023]. These fibrils preferentially deposit in musculoskeletal tissues, including joints, tendons, and bones, leading to Dialysis-Related Amyloidosis (DRA), characterized by carpal tunnel syndrome, bone cysts, and destructive arthropathy [PubMed: 29147140]. Therapeutic interventions focus on reducing circulating B2M levels through high-flux dialysis or adsorption columns like Lixelle, as well as investigating small molecules like doxycycline and monoclonal antibodies that can stabilize the native B2M structure or disrupt existing fibrils [PubMed: 22451547].

Other names

Beta-2-microglobulin amyloidB2M amyloidAbeta2MDialysis-related amyloid

Inhibition of amyloid fibril formation, stabilization of the native monomeric state, or mechanical removal of the precursor protein from circulation.