Pah^em2Xiwan

CRISPR/cas9 endonuclease-mediated homology-directed repair was used to replace a portion of exon 7 with the orthologous human PAH sequence resulting in a non-humanized loss-of-function allele with a 4-bp deletion (GTAA) just distal to the site of an amino acid substitution at position 281 altering proline to leucine (P281L; c.842 C to T) of the phenylalanine hydroxylase (Pah) gene on chromosome 10. (J:344273)