Pathological Investigation of the Pathogenesis and Development of Preventive and Therapeutic Procedures for Amyloidosis

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Amyloidosis refers to a group of protein folding disorders characterized by accumulation of fine amyloid fibrils. The patients of amyloidoses are increasing and it has been longing for the development of new therapeutic and preventive procedures.We used animal models of amyloidosis including mouse AApoAII and AA amyloidosis and revealed that 1) Amyloidosis may be transmitted by prion-like infectious process. We found that amyloid fibrils in the feces and blood/blood cells could serve as self-propagating agents for the instigation and progression of amyloidosis. 2) Activated endoplasmic reticulum (ER) stress signals play an important role both in protecting and damaging tissues associated with amyloid deposition. 3) Deficiency of apoliporptein A-I accelerated amyloidosis.We succeeded in suppressing amyloid deposition in mice by treatment with the C-terminal peptide of type F ApoA-II protein which blocks the active ends of premature amyloid fibrils for self-propagation.