Stmn2^{em8(STMN2*)Lutzy}

CRISPR/cas9 endonuclease-mediated genome editing uses guide RNAs were selected to target intron 1 and insert a modified human SMNT2 exon 2a. The humanized/mutant exon 2a in this strain is 222nt in length and is flanked by 1491 bp and 1548 bp of human genomic DNA, located 5'- and 3'- of exon 2a, respectively. The 19-nt MS2 stem loop contains the sequence 5'-ACATGAGGATCACCCATGT-3' is used to replace the 24-nt TDP-43 binding sequence 5';-TGTGTGAGCATGTGTGCGTGTGTG-3' in the 3';-UTR of the exon2a-containing mouse-human hybrid STMN2 prematurely terminated mRNA transcript. (J:334251)