RESUMO
In congenital stationary night blindness type 2 (CSNB2)-a disorder involving dysfunction of the Cav1.4 Ca2+ channel-visual impairment is relatively mild considering that Cav1.4 mediates synaptic transmission by rod and cone photoreceptors. Here, we addressed this conundrum using a Cav1.4 knockout (KO) mouse and a knock-in (KI) mouse expressing a non-conducting Cav1.4 mutant. Surprisingly, aberrant Cav3 currents were detected in cones of the KI and KO but not wild-type mice. Cone synapses, which fail to develop in KO mice, are present but enlarged in KI mice. Moreover, light responses in cone pathways and photopic visual behavior are preserved in KI but not in KO mice. In CSNB2, we propose that Cav3 channels maintain cone synaptic output provided that the Ca2+-independent role of Cav1.4 in cone synaptogenesis remains intact. Our findings reveal an unexpected form of homeostatic plasticity that relies on a non-canonical role of an ion channel.
