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Toward Establishment of an Animal Model for Familial Amyloidotic Polyneuropathy: Generation and Characterization of Transgenic Mice

  • Hiroyuki Sasaki
  • Masamitsu Nakazato
  • Shigenobu Tone
  • Tetsuyuki Kitamoto
  • Katsuji Yoshioka
  • Hisayuki Matsuo
  • Jun Tateishi
  • Yoshihiro Kato
  • Yoshiyuki Sakaki
Chapter

Abstract

Type I familial amyloidotic polyneuropathy (FAP) results from the systemic accumulation of a plasma transthyretin (TTR) variant with a Val to Met change at position 30. As an approach to clarify the pathological process of this disease, we attempted to produce an animal model by introducing the mutated TTR gene into the mouse germ line. A total of ten transgenic mice carrying the mouse metallothionein-I-human variant TTR fusion gene was generated. To characterize these mice, the integrated fusion gene and its products were analyzed by various methods. The data demonstrate that the introduced genes are actually expressed and the variant TTR is secreted into the serum. These mice should be useful in elucidating the biochemical and biophysical nature of the variant form of TTR and eventually the disease process of type I FAP.

Keywords

Transgenic Mouse Fusion Gene Amyloid Fibril Cyanogen Bromide Familial Amyloidotic Polyneuropathy 
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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Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Hiroyuki Sasaki
    • 1
  • Masamitsu Nakazato
    • 2
  • Shigenobu Tone
    • 3
  • Tetsuyuki Kitamoto
    • 4
  • Katsuji Yoshioka
    • 1
  • Hisayuki Matsuo
    • 2
  • Jun Tateishi
    • 4
  • Yoshihiro Kato
    • 3
  • Yoshiyuki Sakaki
    • 1
  1. 1.Research Laboratory for Genetic InformationKyushu UniversityFukuoka 812Japan
  2. 2.Department of BiochemistryMiyazaki Medical CollegeKiyotakeJapan
  3. 3.Mitsubishi-Kasei Institute of Life SciencesMachida 197Japan
  4. 4.Department of Pathology, Neurological InstituteKyushu UniversityFukuoka 812Japan

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