Targeting Amyloid Fibrils by Passive Immunotherapy in Systemic Amyloidosis.

ABSTRACT: Systemic amyloidoses are characterized by the unrelenting deposition of autologous proteins as highly ordered fibrils in target organs. The ensuing, potentially fatal organ dysfunction is the result of the combined damage caused by the proteotoxic effect of prefibrillar species and by the cytotoxicity and the structural alterations produced by the amyloid fibrils. Current therapy is focused on eliminating the amyloid protein, thus extinguishing the amyloid cascade at its origin. While this approach may end the cell damage caused by prefibrillar aggregates and prevent further amyloid accumulation, the noxious effects of the amyloid fibrils persist and may hamper the recovery of organ function, which is the ultimate goal of therapy as it is necessary to improve the quality of life and extend

SUBMITTER: Nuvolone M 

PROVIDER: S-EPMC9466354 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature