Tg(Thy1-SERPINI1*G392E)333Icka

The promoter was used to drive neuron-specific expression of the human cDNA containing nucleotide substitutions that result in the amino acid substitution of glutamic acid for glycine at position 392 (G392E). This mutation was originally discovered in patients with familial encephalopathy with neuroserpin inclusion bodies (FENIB). Three lines were created (333, 42, 115) with line 333 expressing at much higher levels than lines 42 and 115. (J:144649)