Exon 6 was replaced wiht on in which a point mutation (C to A) results in the amino acid substitution of glutamine for proline at position 141 (P141Q). Cre-mediate recombination removed the floxed neomycin resistance cassette inserted downstream of the modified exon. The modification mimics one identified in human patients with Autosomal-Dominant Dentin Dysplasia Type I. Real-time PCR confirmed reduced transcript expression in the underjaw tissue in homozygotes. (J:240559)