ABSTRACT
Given the fact that approximately 90% of plasma transthyretin (TTR) is produced by the liver, liver transplantation (LT) has been used to abolish the production of most of the mutant TTR in familial amyloid polyneuropathy (FAP). As the shortage of donor livers has been the rate-limiting factor in the expansion of LT, domino liver transplantation (DLT), has been performed; in this procedure FAP patients receive and sequentially donate grafts to other recipients. However DLT involves specific ethical problems namely, the graft of a liver producing a pathogenic protein. This chapter studies the presence of de novo amyloid deposition and toxicity in the skin and nerve of recipients of FAP livers up to 7 years after DLT. De novo amyloidosis occurs in the skin and nerve of recipients of FAP livers after DLT. Sites related to TTR deposition did not display signs of oxidative stress such as increase in 3-NT epitopes probably due to the reduced amount of deposited TTR.
