Ando Yukio, Terazaki Hisayasu, Haraoka Katsuki, Tajiri Takahiro, Nakamura Masaaki, Obayashi Konen, Misumi Shogo, Shoji Shozo, Hata Kanako, Nakagawa Kazuko, Ishizaki Takashi, Uemoto Shinji, Inomata Yukihiro, Tanaka Koichi, Okabe Hiroaki
Department of Laboratory Medicine, Kumamoto University School of Medicine, Kumamoto 860-0811, Japan.
Transplantation. 2002 Mar 15;73(5):751-5. doi: 10.1097/00007890-200203150-00016.
Recently, sequential liver transplantation has been performed with an explanted liver from a patient with familial amyloidotic polyneuropathy (FAP) because of the shortage of donors. However, metabolism of amyloidogenic transthyretin (ATTR), the pathogenic protein of FAP, has not been well studied in patients who have undergone sequential liver transplantation. The purpose of this study was to examine the changes in serum ATTR levels and to investigate the presence of an autoantibody in patients who underwent sequential liver transplantation with an explanted organ from a patient with heterozygotic FAP (FAP ATTR Val30Met).
This was a case study performed at the Kumamoto University School of Medicine, Kumamoto, Japan, and Kyoto University School of Medicine, Kyoto, Japan. Intervention occurred by sequential liver transplantation with an explanted FAP patient's liver. Levels of normal TTR and ATTR in the two patients who received the transplanted liver were analyzed by means of an enzyme-linked immunosorbent assay (ELISA) and a matrix-assisted laser desorption/time-of-flight mass spectrometry. In addition, the presence of an autoantibody against ATTR Val30Met was evaluated via ELISA using purified ATTR Val30Met from homozygotic FAP patients' sera.
After the operation, the variant TTR levels were unexpectedly lower than levels of normal TTR in serum samples from patients with a transplanted liver from the FAP patient. An autoantibody against the variant TTR was detected on day 3 after the operation in the serum of those patients and continued to be present for at least 2 months after the operation.
An autoantibody against the variant TTR may reduce the serum levels of variant TTR. Although the antibody may play a beneficial role in reducing the pathogenic protein, the long-term effect of the antibody must be investigated further.
最近，由于供体短缺，已使用来自家族性淀粉样多神经病（FAP）患者的移植肝进行序贯肝移植。然而，FAP的致病蛋白淀粉样前体蛋白（ATTR）在接受序贯肝移植的患者中的代谢情况尚未得到充分研究。本研究的目的是检测接受来自杂合子FAP（FAP ATTR Val30Met）患者的移植肝进行序贯肝移植的患者血清ATTR水平的变化，并调查自身抗体的存在情况。
这是一项在日本熊本熊本大学医学院和日本京都京都大学医学院进行的病例研究。干预措施为使用FAP患者的移植肝进行序贯肝移植。通过酶联免疫吸附测定（ELISA）和基质辅助激光解吸/飞行时间质谱法分析接受移植肝的两名患者中正常TTR和ATTR的水平。此外，使用来自纯合子FAP患者血清的纯化ATTR Val30Met通过ELISA评估针对ATTR Val30Met的自身抗体的存在情况。
手术后，来自接受FAP患者移植肝的患者血清样本中，变异型TTR水平意外低于正常TTR水平。这些患者术后第3天在血清中检测到针对变异型TTR的自身抗体，并且在术后至少2个月内持续存在。
针对变异型TTR的自身抗体可能会降低血清中变异型TTR的水平。尽管该抗体可能在减少致病蛋白方面发挥有益作用，但必须进一步研究该抗体的长期效果。
