Fig. 1: Establishment of Pou4f3WT/Q113* transgenic mice.
From: Optimized in vivo base editing restores auditory function in a DFNA15 mouse model
A The amino acid substitution in Pou4f3 (c.337C > T, p.Q113*) mutant mice occurs at a location (red) that is highly conserved in vertebrate evolutionary clades such as humans, monkeys, and zebrafish. B Representative Sanger sequencing results of the Pou4f3 allele in WT, Pou4f3WT/ Q113* and Pou4f3Q113*/ Q113* mice. C The results of click ABR thresholds in Pou4f3WT/Q113* mice (red) and WT mice (black). WT vs Pou4f3WT/Q113*: p = 0.476179 (1 month), p = 0.105612 (2 months), p = 0.037513 (3 months), p = 0.001093 (4 months). D The results of TB-ABR thresholds in Pou4f3WT/Q113* mice (red) and WT mice (black). (WT vs Pou4f3WT/ Q113*: 1 month, p = 0.861927 (4k), p = 0.040231 (8k), p = 0.191765 (12k), p = 0.006708 (16k), p = 0.037929 (24k), p = 0.035894 (32k); 2 months, p = 0.001634 (4k), p = 0.082764 (8k), p = 0.004614 (12k), p = 0.000023 (16k), p = 0.000192 (24k), p = 0.000097 (32k); 3 months, p = 0.174963 (4k), p = 0.012478 (8k), p < 0.000001 (12k), p = 0.000007 (16k), p < 0.000001 (24k), p = 0.001515 (32k); 4 months, p = 0.001956 (4k), p = 0.001110 (8k), p = 0.000275 (12k), p < 0.000001 (16k), p < 0.000001 (24k), p = 0.000015 (32k). E Representative immunofluorescence images of hair cells from WT and the Pou4f3WT/Q113* mice at P90, n = 4 independent experiments with similar results. Myo7a (green): hair cell marker. ‘*’ Indicates the missing hair cells. Scale bar, 20 μm. F Quantification of cochlear hair cells corresponding to (F). n = 4, WT vs Pou4f3WT/Q113*: in OHCs, p > 0.999999 (apex), p = 0.002452 (middle), p = 0.000050 (base), in IHCs, p = 0.113532 (apex), p = 0.097160 (middle), p = 0.097160 (base). G Representative SEM micrographs of OHCs and IHCs in the cochleae from WT and Pou4f3WT/Q113* mice at P30 and P60, n = 3 independent experiments with similar results. Scale bar, 5 μm. Data in (C, D, F) are represented as mean ± SEM. Statistical significance was determined by unpaired two-tailed Student’s t-tests (*p < 0.05, **p < 0.01, ***p < 0.001, ns no significant difference). Source data are provided as a Source data file.
