Tg(TTR-V30M)^dl14Imeg

This transgenic line was derived from Tg(TTR-V30M)14Imeg by spontaneous loss of all except one of an estimated five to six tandem copies of the transgene present in the latter line; approximately 1 kb of genomic DNA downstream of the transgene is also deleted. The transgene comprises a 7.6-kilobase genomic DNA fragment containing the entire human transthyretin gene, with a mutation causing substitution of methionine for valine at amino acid position 30, and approximately 0.6 kb of upstream sequence isolated from a patient with familial amyloidotic polyneuropathy. In contrast to mice of the original line 14, mice of the present line do not have detectable levels of human transthyretin in serum nor of human TTR mRNA in the face at any developmental stage. (J:81049)