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Mouse Models of Transthyretin Amyloidosis

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Book cover Recent Advances in Transthyretin Evolution, Structure and Biological Functions

Abstract

Transthyretin (TTR) amyloidosis is caused by mutations in the TTR gene. Several observations, however, suggest the presence of factors, other than a mutation in the TTR gene, which affect TTR amyloid deposition. Although liver transplantation is the only curative treatment for TTR amyloidosis, its donor pool faces shortage, and TTR amyloid deposition continues in many patients after transplantation. Thus, some effective therapeutic strategies other than liver transplantation need to be developed. Mouse models of TTR amyloidosis would facilitate defining factors that accelerate amyloid deposition and would aid in developing effective treatments. Here, we summarize studies of transgenic mouse models of TTR amyloidosis in which questions were addressed about the role of various risk factors in the molecular pathogenesis of this intractable disease.

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Acknowledgments

This work was supported by grants from the Ministry of Education, Culture, Sports, Science and Technology, Japan; Grants-in-aids for Scientific Research (to SM); and by grants from the Ministry of Health, Labour and Welfare, Japan; the Amyloidosis Research Committee, Surveys and Research on Specific Diseases (to SM). We thank all our collaborators for their invaluable help and contributions.

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Authors and Affiliations

  1. Department of Biochemistry, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, 1110 Shimokato, Chuo, Yamanashi, 409-3898, Japan

    Sadahiro Ito & Shuichiro Maeda

  2. Center for Life Science Research, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, 1110 Shimokato, Chuo, Yamanashi, 409-3898, Japan

    Sadahiro Ito

Authors
  1. Sadahiro Ito
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  2. Shuichiro Maeda
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Correspondence to Shuichiro Maeda .

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Editors and Affiliations

  1. School of Medical Sciences, RMIT University, Bundoora, VIC 3083, Bundoora West Campus, Australia

    Samantha J. Richardson

  2. Medical Research Institute, Hauptman-Woodward, 700 Elliott Street, Buffalo, NY, 14203, USA

    Vivian Cody

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Ito, S., Maeda, S. (2009). Mouse Models of Transthyretin Amyloidosis. In: Richardson, S.J., Cody, V. (eds) Recent Advances in Transthyretin Evolution, Structure and Biological Functions. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-00646-3_16

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