A study of the pathomechanism of amyloid fibril turnover in FAP patients after liver transplantation

本研究旨在阐明家族性淀粉样多发性神经病（FAP）患者肝移植后淀粉样纤维沉积清除的病理机制。对接受肝移植的FAP患者胃粘膜中沉积的淀粉样纤维的研究表明，肝移植前后沉积的淀粉样纤维的量没有明显差异。然而，沉积的淀粉样蛋白中野生型甲状腺素运载蛋白（TTR）的组成比显著增加。我们的研究结果表明，野生型TTR的沉积和由变体和野生型TTR组成的淀粉样纤维的清除（淀粉样蛋白周转）总是发生在FAP患者移植后阶段的每个器官中。

The aim of this study is to elucidate the pathomechaism of clearance of deposited amyloid fibrils in patients with familial amyloid polyneuropathy (FAP) with having undergone liver transplantation. On investigation of deposited amyloid fibrils in the gastric mucosa in FAP patients who had undergone liver transplantation, the amount of deposited amyloid was not markedly different between before and after liver transplantation. However, the composition ratio of the wild-type transthyretin (TTR) in deposited amyloid significantly increased. Our results suggest that the deposition of wild-type TTR and clearance of amyloid fibrils composed with variant and wild-type TTR (amyloid turnover) always occur in every organ in post -transplant stage of FAP patients.