Systemic analysis of amyloidosis using animal models

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Amyloidoses are protein structural disorders characterized by the extracellular deposition of insoluble amyloid fibrils resulting from abnormal conformational changes. We have developed a new analytical system for amyloid fibril formation from synthetic peptides in test tubes and several new mouse strains for studying various kinds of amyloidoses. Using these in vitro and in vivo systems, systemic analysis of amyloidosis was performed to elucidate the pathogenesis of transmission of amyloidosis. We found 1) new transmission routes through feces and skeletal muscle, 2) new therapeutic targets including peptides inhibiting fibril formation, heat shock transcription factor 1 (HSF1) and apolipoprotein A-I, and 3) several newly developed mouse strains variable for amyloidosis researches.