Tpp1^tm1.1Dprc

The targeting vector is designed to insert an FRT-flanked neomycin (neo) resistance cassette downstream of exon 6 of the gene. A C to T transition was introduced in exon 6, corresponding to human amino acid 208, resulting in a nonsense mutation, R207X, commonly found in patients with late infantile Neuronal Ceroid Lipofuscinoses (NCL) or Batten disease. Flp-mediated recombination removed the FRT-flanked neo cassette. (J:241344)