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English: FIGURE 2. Mechanisms-driving TTR amyloidogenesis and different therapeutic targets for the treatment of ATTR amyloidosis. Tetramer destabilization is widely accepted as a rate-limiting step for the development of amyloid fibrils. However, TTR proteolysis has been increasingly suggested as an alternative mechanism contributing to amyloid formation. Several pharmacological agents have been implicated in the treatment of ATTR amyloidosis, from inhibitors of TTR synthesis, tetramer stabilizers, inhibitors of amyloid formation and even disruptors of formed fibrils. The modulation of TTR proteolysis may also be helpful for the treatment of ATTR amyloidosis. Despite the protease responsible for this process has not yet been identified, its specific cleavage patterns suggest that it could be a trypsin-like serine protease. Accordingly, pharmacological molecules targeting lysine residues, as well as physiological serine protease inhibitors may be act as modulators of TTR proteolysis, consequently inhibiting amyloid formation.