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Abnormal Transthyretin in Familial Amyloidotic Polyneuropathy in Japan and Sweden

  • Masayuki Tanaka
  • Masamitsu Nakazato
  • Teruyuki Kurihara
  • Shigeru Matsukura
  • Kenji Kangawa
  • Hisayuki Matsuo
Chapter

Abstract

In order to clarify the biochemical abnormalities of familial amyloidotic polyneuropathy in northern Sweden and in various districts of Japan, amino acid sequencing study and radioimmunoassay were perfomed. The results indicated that both Japanese and Swedish FAP patients have systemic deposition of a variant transthyretin with a methionine-for-valine substitution at position 30. This variant transthyretin was present in the serum and the cerebrospinal fluid of FAP patients and half of their children. The variant transthyretin was detected in ten elderly asymptomatic carriers of FAP whose ages are far beyond the usual age of onset of the disease.

Keywords

High Performance Liquid Chromatography Sporadic Case Amyloid Fibril Biochemical Abnormality Cyanogen Bromide 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    Nomenclature Committee of IUB (NC-IUB) IUB-IUPAC Joint Commission on Biochemical Nomenclature (JCBN), J Biol Chem 256:12 (1981).Google Scholar
  2. 2.
    S. Tawara, M. Nakazato, K. Kangawa, et al, Identification of amyloid prealbumin variant in familial amyloidotic polyneuropathy (Japanese type), Biochem Biophys Res Commun. 116:880 (1983).CrossRefGoogle Scholar
  3. 3.
    M. Nakazato, K. Kangawa, N. Minamino, et al, Identification of a prealbumin variant in the serum of a Japanese patient with familial amyloidotic polyneuropathy, Biochem Biophys Res Commun. 122:712 (1984).CrossRefGoogle Scholar
  4. 4.
    M. Nakazato, K. Kangawa, N. Minamino, et al, Radioimmunoassay for detecting abnormal prealbumin in the serum for diagnosis of familial amyloidotic polyneuropathy (Japanese type), Biochem Biophys Res Commun. 122:719 (1984).CrossRefGoogle Scholar
  5. 5.
    M. Nakazato, T. Kurihara, K. Kangawa, et al, Diagnostic radioimmunoassay for familial amyloidotic polyneuropathy, Lancet ii:1274 (1984).CrossRefGoogle Scholar
  6. 6.
    M. Nakazato, T. Kurihara, S. Matsukura, et al, Diagnostic radioimmunoassay for familial amyloidotic polyneuropathy before clinical onset, J Clin Invest. 77:1699 (1986).CrossRefGoogle Scholar
  7. 7.
    M. Nakazato, H. Sasaki, H. Furuya, et al, Biochemical and genetic characterization of type I familial amyloidotic polyneuropathy, Ann Neurol. 21:596 (1987).CrossRefGoogle Scholar
  8. 8.
    M. Nakazato, L. Steen, G. Holmgren, et al, Structurally abnormal transthyretin causing familial amyloidotic polyneuropathy in Sweden, Clin Chem Acta. 167:341 (1987).CrossRefGoogle Scholar
  9. 9.
    M. Nakazato, K. Kangawa, T. Kurihara, et al, Variant transthyretin in cerebrospinal fluid in familial amyloidotic polyneuropathy, J Neurol Sci. 79:111 (1987).CrossRefGoogle Scholar
  10. 10.
    M. Nakazato, M. Tanaka, Y. Yamamura, et al, Abnormal transthyretin in asymptomatic relatives in familial amyloidotic polyneuropathy, Arch Neurol. (in press).Google Scholar
  11. 11.
    M. Tanaka, N. Nakazato, T. Kurihara, et al, Biochemical characterization of familial amyloidotic polyneuropathy in various districts of Japan, Jpn J Med. 26:189 (1987).CrossRefGoogle Scholar
  12. 12.
    F. E. Dwulet, and M. D. Benson, Primary structure of an amyloid prealbumin and its plasma precursor in a heredofamilial polyneuropathy of Swedish origin, Proc Natl Acad Sci USA. 81:694 (1984).CrossRefGoogle Scholar
  13. 13.
    H. Sasaki, Y. Sakaki, H. Matsuo, et al, Diagnosis of familial amyloidotic polyneuropathy by recombinant DNA techniques, Biochem Biophys Res Commun. 125:636 (1984).CrossRefGoogle Scholar
  14. 14.
    S. Mita, S. Maeda, M. Ide, et al, Familial amyloidotic polyneuropathy diagnosed by cloned human prealbumin cDNA, Neurology. 36:298 (1986).CrossRefGoogle Scholar
  15. 15.
    S. Ikeda, N. Hanyu, M. Hongo, et al, Hereditary generalized amyloidoses with polyneuropathy, Clinicopathological study of sixty-five Japanese patients, Brain. 110:315 (1987).CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Masayuki Tanaka
    • 1
  • Masamitsu Nakazato
    • 1
  • Teruyuki Kurihara
    • 1
  • Shigeru Matsukura
    • 1
  • Kenji Kangawa
    • 1
    • 2
  • Hisayuki Matsuo
    • 1
    • 2
  1. 1.Departments of MedicineMiyazaki Medical CollegeKiyotake, MiyazakiJapan
  2. 2.Departments of BiochemistryMiyazaki Medical CollegeKiyotake, MiyazakiJapan

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