Figure 10. AAV2/8-mediated delivery of Clrn1 in Clrn1^ex4–/– mice preserves synapse ribbon structure and function.

(A) The re-expression of clarin-1 at the IHC synapse (P15–P18) in Clrn1^ex4–/– IHCs led to normal Ca²⁺ currents, kinetics, and Ca²⁺ efficiency of exocytosis (Supplemental Table 3). *P < 0.05 (2-way ANOVA). (B) The virus-mediated expression of clarin-1 restored the tight clustering of Ca²⁺ channels in the IHC active zone, as shown by the line scan intensity profiles of Ca_v1.3- and ribeye-immunoreactive patches for 2 ribbons (images representative of 3 mice).