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Use of mouse models to analyse the molecular bases of familial amyloidotic polyneuropathy type I
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文摘
To investigate the molecular bases of familial amyloidotic polyneuropathy (FAP) type I, we generated transgenic mice carrying the human mutant ttr gene (ttr met30) that is responsible for FAP type I. In these mice, human TTR was deposited as amyloid fibrils in various tissues. The transgenic mice, while useful in elucidating the mechanism of amyloid deposition in FAP, do not, however, develop peripheral neuropathy.

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