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To generate this mutant allele, an ACN targeting vector was designed to insert (by site-directed mutagenesis) a single amino acid substitution of arginine to proline at codon 1278 (p.R1278P). The ACN cassette contains a neomycin resistance gene and cre recombinase gene under the control of the sperm-specific angiotensin-converting enzyme promoter and is flanked by loxP sites. Cre-mediated recombination during spermatogenesis of chimeric males removes the cassette leaving a single loxP site in intron 27. The mutation is the equivalent of the human p.R1276P mutation. (J:94077)