Toward Establishment of an Animal Model for Familial Amyloidotic Polyneuropathy: Generation and Characterization of Transgenic Mice
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.
KeywordsTransgenic Mouse Fusion Gene Amyloid Fibril Cyanogen Bromide Familial Amyloidotic Polyneuropathy
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