This Article Order Full text via Infotrieve Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yi, S. Articles by Araki, S. Search for Related Content PubMed PubMed Citation Articles by Yi, S. Articles by Araki, S.

American Journal of Pathology, Vol 138, 403-412, Copyright © 1991 by American Society for Investigative Pathology

REGULAR ARTICLES

Systemic amyloidosis in transgenic mice carrying the human mutant transthyretin (Met30) gene. Pathologic similarity to human familial amyloidotic polyneuropathy, type I

S Yi, K Takahashi, M Naito, F Tashiro, S Wakasugi, S Maeda, K Shimada, K Yamamura and S Araki
Second Department of Pathology, Kumamoto University Medical School, Japan.

To analyze the pathologic processes of amyloid deposition in type I familial amyloidotic polyneuropathy (FAP), mice were made transgenic by introducing the human mutant transthyretin (TTR) gene. In these transgenic mice, amyloid deposition started in the gastrointestinal tract, cardiovascular system, and kidneys 6 months after birth and extended to various other organs and tissues with advancing age. At age 24 months, the pattern of amyloid deposition was similar to that observed in human autopsy cases of FAP, except for its absence in the choroid plexus and in the peripheral and autonomic nervous systems. Amyloid deposition was shown to be composed of human mutant TTR and, in addition, mouse serum amyloid P component. These results clearly indicate that human variant TTR produced in transgenic mice deposits is a major component of amyloid fibrils in various organs and tissues. Thus this animal model is useful for analyzing how amyloid deposition initiates and proceeds in FAP.

This article has been cited by other articles:

				R. Snanoudj, A. Durrbach, E. Gauthier, D. Adams, D. Samuel, S. Ferlicot, P. Bedossa, A. Prigent, H. Bismuth, and B. Charpentier Changes in renal function in patients with familial amyloid polyneuropathy treated with orthotopic liver transplantation Nephrol. Dial. Transplant., July 1, 2004; 19(7): 1779 - 1785. [Abstract] [Full Text] [PDF]

				Y. Takaoka, M. Ohta, K. Miyakawa, O. Nakamura, M. Suzuki, K. Takahashi, K.-i. Yamamura, and Y. Sakaki Cysteine 10 Is a Key Residue in Amyloidogenesis of Human Transthyretin Val30Met Am. J. Pathol., January 1, 2004; 164(1): 337 - 345. [Abstract] [Full Text] [PDF]

				H. Koike, K.-i. Misu, S.-i. Ikeda, Y. Ando, M. Nakazato, E. Ando, M. Yamamoto, N. Hattori, G. Sobue, and for the Study Group for Hereditary Neuropathy in J Type I (Transthyretin Met30) Familial Amyloid Polyneuropathy in Japan: Early- vs Late-Onset Form Arch Neurol, November 1, 2002; 59(11): 1771 - 1776. [Abstract] [Full Text] [PDF]

				M. M. Sousa, R. Fernandes, J. A. Palha, A. Taboada, P. Vieira, and M. J. Saraiva Evidence for Early Cytotoxic Aggregates in Transgenic Mice for Human Transthyretin Leu55Pro Am. J. Pathol., November 1, 2002; 161(5): 1935 - 1948. [Abstract] [Full Text] [PDF]

				K.-i. Misu, N. Hattori, M. Nagamatsu, S.-i. Ikeda, Y. Ando, M. Nakazato, Y.-i. Takei, N. Hanyu, Y. Usui, F. Tanaka, et al. Late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy) unrelated to endemic focus in Japan: Clinicopathological and genetic features Brain, October 1, 1999; 122(10): 1951 - 1962. [Abstract] [Full Text] [PDF]