Fig. 1
Prestin expression and function in NPC1-KO mice. a-b Immunofluorescent images of OHCs from WT and NPC1-KO mice showing normal prestin expression. Representative images from the middle of the cochlea in a P49 female WT (a) and P49 male NPC1-KO (b), stained with anti-N-mprestin (green) and phalloidin-Alexa 546 (red) showing normal prestin expression. Scale bars, 100 μm. c. 3D reconstruction of z-stack images of OHCs from P49 male WT and female NPC1-KO cochleae (scale bar, 5 μm), showing the characteristic cylindrical staining of prestin at the lateral membrane. Red, phalloidin (actin); Blue, nuclei. d-g. NLC measurements of OHCs isolated from two female P52 WT and one female P78 and two male P29 NPC1-KO mice. d Individual recordings of NLC are shown in gray (WT) and pink (NPC1-KO), and the solid black (WT) and red (NPC1-KO) lines indicate fitted curves. e Charge density (CD). Mean ± s.d. are: WT, 135.5 ± 6.5 fC/pF (n = 9); NPC1-KO, 138.6 ± 16.2 fC/pF (n = 8). F alpha. Mean ± s.d. are: WT, 0.028 ± 0.002 mV− 1 (n = 9); NPC1-KO, 0.031 ± 0.003 mV− 1 (n = 8). g Vpkcm. Mean ± s.d. are: WT, − 87.41 ± 9.3 mV (n = 9); NPC1-KO, − 60.57 ± 12.0 mV (n = 8). P values are indicated in each figure (unpaired t-test)