Figure 1. Hypothetical model depicting CLN3-regulation of calsenilin function.

Under low Ca²⁺ conditions, CLN3 can bind calsenilin which is protective from Ca²⁺-induced cell death and calsenilin regulation of Kv4.2. In high Ca²⁺ conditions, calsenilin binds Ca²⁺ and dissociates from CLN3, where it is then able to interact with other proteins like Kv4.2 to influence their trafficking and/or function. A pool of CLN3 localized at the plasma membrane (PM) (not shown) may alternatively be involved in Kv4.2 channel regulation. Cal=calsenilin, Lys=lysosome