Table 1. Mutations in numerous cilia genes leads to resistance in neomycin-induced hair cell death.
Wild-Type Sibling | Mutant | ||||||
---|---|---|---|---|---|---|---|
Gene | Control | 200 μM Neomycin | % HCs Remaining | Control | 200 μM Neomycin | % HCs Remaining | |
Ciliary axoneme and intraflagellar transport | |||||||
dync2h1 (w46) | 12.13 ± 1.15 | 1.05 ± 0.33 | 8.65 | 8.65 ± 1.27 | 5.63 ± 1.26 | 65.13 | |
P < 0.0001 | P < 0.0001 | ||||||
wdr35 (w150) | 11.37 ± 0.92 | 1.84 ± 0.63 | 16.18 | 10.10 ± 0.89 | 6.17 ± 1.41 | 61.89 | |
P = 0.0002 | P < 0.0001 | ||||||
ift88 (tz288) | 12.38 ± 1.78 | 1.17 ± 0.71 | 9.42 | 9.27 ± 0.65 | 4.43 ± 1.70 | 47.74 | |
P < 0.0001 | P < 0.0001 | ||||||
traf3ip1 (tp49d) | 11.6 ± 1.43 | 1.08 ± 0.55 | 9.34 | 6.73 ± 0.79 | 4.78 ± 0.69 | 71.04 | |
P < 0.0001 | P < 0.0001 | ||||||
arl13b (hi459Tg) | 11.78 ± 1.54 | 1.65 ± 0.85 | 14.00 | 11.92 ± 1.01 | 1.30 ± 0.37 | 10.88 | |
P = 0.9556 | P = 0.7145 | ||||||
Transition zone | |||||||
cc2d2a (w38) | 11.10 ± 1.72 | 1.53 ± 0.69 | 13.78 | 12.33 ± 1.93 | 3.68 ± 0.47 | 29.85 | |
P = 0.0975 | P = 0.0023 | ||||||
cc2d2a (w123) | 12.78 ± 2.5 | 1.17 ± 0.38 | 9.13 | 12.73 ± 2.24 | 3.05 ± 0.48 | 23.95 | |
P = 0.9973 | P = 0.0373 | ||||||
mks1 (w152) | 12.34 ± 0.86 | 1.26 ± 0.46 | 10.21 | 12.17 ± 0.83 | 2.88 ± 0.65 | 23.66 | |
P = 0.6768 | P < 0.0001 | ||||||
cep290 (fh378) | 12.30 ± 1.02 | 1.44 ± 0.34 | 11.70 | 12.19 ± 1.04 | 2.24 ± 0.88 | 18.34 | |
P = 0.7217 | P = 0.0244 |
Numbers are average number of hair cells/neuromast ± SD. Significance was calculated using an ANOVA (analysis of variance) and Šídák multiple comparisons test. n = 10 for dync2h1, ift88, traf3ip, arl13b, cc2d2aw38, and cc2d2aw123, n = 16 for cep290, n = 31–37 for wild-type siblings, and n = 8–14 for mutants of wdr35 and mks1. HCs, hair cells.