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THE AMYLOID RELATED TRANSTHYRETIN METABOLISM POST DOMINO LIVER TRANSPLANTATION

Research Project

Project/Area Number 13670638
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionSHINSHU UNIVERSITY

Principal Investigator

TAKEI Yo-ichi  SHINSHU UNIVERSITY, assistant, 医学部, 助手 (90273086)

Co-Investigator(Kenkyū-buntansha) IKEDA Shu-ichi  SHINSHU UNIVERSITY, professor, 医学部, 教授 (60135134)
TOKUDA Takahiko  SHINSHU UNIVERSITY, lecturer, 医学部, 助教授 (80242692)
Project Period (FY) 2001 – 2002
Project Status Completed (Fiscal Year 2002)
Budget Amount *help
¥3,400,000 (Direct Cost: ¥3,400,000)
Fiscal Year 2002: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 2001: ¥2,600,000 (Direct Cost: ¥2,600,000)
Keywordsfamilial amyloid polyneuropathy / transthyretin / domino liver transplantation / mass spectrometry / transthyretin monomer / 肝移植 / ドミノ移植 / モノマー
Research Abstract

We have diagnosed 20 familial amyloid polyneuropathy (FAP) patients and 5 FAP carriers since 2001. Most of these patients were diagnosed as having Val30Met transthyretin (TTR) mutation. We found a rare case with carpal tunnel syndrome and severe cardiac amyloidosis, whose ATTR Tyr69Ile was found to be caused by double-nucleotide substitution at codon 69.
Twenty liver transplantations have been performed since 1993 in patients with FAP, including 5 domino liver transplantations. After the operation the serum concentration of mutant type TTR is increased promptly in domino recipients. Serial examinations of clinical findings and abdominal fat aspiration biopsy were performed for them after the operation. However, no sign of polyneuropathy, autonomic neuropathy and amyloid deposition was detected respectively. In FAP patients, mass spectrometry showed that the variant monomer was markedly decreased compared with the wild-type TTR monomer. In domino recipients who were transplanted FAP liver, variant monomer was not decreased. These findings suggest that the free variant TTR monomer is unstable in serum, and that it aggregates in deposits in various organs in FAP patients. However, in domino recipient amyloid deposition or amyloid fibril formation has not yet started within two or three years after operation. It is suggested that free variant TTR monomer is useful to know the beginning of amyloid deposition.

Report

(3 results)
  • 2002 Annual Research Report   Final Research Report Summary
  • 2001 Annual Research Report
  • Research Products

    (16 results)

All Other

All Publications (16 results)

  • [Publications] Takei Y, Hattori T, Gono T, Tokuda T, Saitoh S, Hoshii Y, Ikeda S.: "Senile systemic amyloidosis presenting as bilateral carpal tunnel syndrome"Amyloid : The Journal of Protein Folding Disorders. 9(4). 252-255 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Ikegami T, Kawasaki S, Ohno Y, Chisuwa H, Urata K, Nakazawa Y, Hashikura Y, Terada M, Miyagawa S, Takei Y, Tokuda T, Ikeda S.: "Temporary auxiliary liver transplantation from a living donor to an adult recipient with familial amyloid polyneuropathy"Transplantation. 73(4). 628-630 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Owa M, Takei Y, Hashikura Y, Kawasaki S, Koyama M, Ikeda S.: "Recurrent cerebral embolism in a familial amyloid polyneuropathy patient who received partial liver transplantation from living donor"Intern Med. 40(3). 259-264 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 池田修一, 武井洋一, 服部健, 森田洋: "家族性アミロイドポリニューロパチー(FAP)肝移植後の末梢神経病変"末梢神経. 12(1). 45-48 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Takei Y, Hattori T, Yazaki M, Tokuda T, Urasawa N, Kanai S, Ikeda S.: "Transthyretin Tyr69-to-Ile mutation (double-nucleotide substitution in codon 69) in a Japanese familial amyloidosis patient with cardiomyopathy and carpal tunnel syndrome"Amyloid : The Journal of Protein Folding Disorders. (In press). (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Ikeda S, Takei Y, Hashikura Y.: "Liver transplantation as treatment for neucrological disorders"Expert Review of Neurotherapeutics. (In press). (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Takei Y, Hattori T, Gono T, Tokuda T, Saitoh S, Hoshii Y, and Ikeda S.: "Senile systemic amyloidoeis presenting as bilateral carpal tunnel syndrome."Amyloid. 9(4). 252-255 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Ikegami T, Kawasaki S, Ohno Y, Chisuwa H, Urata K, Nakazawa Y, Hashikura Y, Terada M, Miyagawa S, Takei Y, Tokuda T, and Ikeda S.: "Temporary auxiliary liver transplantation from a living donor to an adult recipient with familial amyloid polyneuropathy."Transplantation. 73(4). 628-630 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Owa M, Takei Y, Hashikura Y, Kawasaki S, Koyama M, Ikeda S: "Recurrent cerebral embolism in a familial amyloid polyneuropathy patient who received partial liver transplantation from a living donor."Intern Med. 40(3). 259-264 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Ikeda S, Takei Y, Hattori T, Morita Y.: "Peripheral nerve function in patients with familial amyloid polyneuropathy (FAP) after liver transplantation, (in Japanese)"Japanese Journal of Peripheral Nerve. 12(1). 45-48 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Takei Y, Hattori T, Yazaki M, Tokuda T, Urasawa N, Kanai S, and Ikeda S: "Transthyretin Tyr69-to-Ile mutation (double-nucleotide substitution in codon 69) in a Japanese familial amyloidosis patient with cardiomyopathy and carpal tunnel syndrome"Amyloid. (in press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Ikeda S, Takei Y, Hashikura Y.: "Liver transplantation as treatment for neurological disorders. Expert Review of Neurotherapeutics"Expert Review of Neurotherapeutics. (in press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Takei Y.et al.: "Senile systemic amyloidosis presenting as bilateral carpal tunnel syndrome"Amyloid : The Journal of Protein Folding Disorders. 9(4). 252-255 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Ikegami T, Takei Y et al.: "Temporary auxiliary liver transplantation from a living donor to an adult recipient with familial amyloid polyneuropathy"Transplantation. 73(4). 628-630 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Takei Y.et al.: "Transthyretin Tyr69-to-Tle mutation (double-nucleotide substitution in codon 69) in a Japanese familial amyloidosis patient with cardiomyopathy and carpal tunnel syndrome"Amyloid : THE Journal of Protein Folding Disorders. (in press). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Ikeda S, Takei Y.et al.: "Liver transplantation as treatment for neurological disorders"Expert Review of Neurotherapeutics. (in press). (2003)

    • Related Report
      2002 Annual Research Report

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Published: 2001-04-01   Modified: 2016-04-21  

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