CLN5 and juvenile neuronal ceroid lipofuscinosis: Our study, involving a unique human neuronal model mimicking CLN5 Batten disease with isogenic controls, revealed (1) our capability to model Batten disease in a dish using CRISPRi and iPSC-derived human neurons; (2) that >90% inhibition of CLN5 expression was achieved using CRISPRi in iPSC-derived human neurons; and that CLN5-deficient human neurons manifest defects in (3) acidic organelles (lysosomes), (4) lysosomal enzyme activity, and (5) lysosomal movement.