The CLN3Δex7/8 miniswine model recapitulates many human phenotypes of CLN3 disease, including consistent retinal degeneration and gait abnormalities, as well as classic Batten disease pathologies, such as storage material accumulation, glial activation, photoreceptor loss, and calbindin interneuron loss in the somatosensory cortex and motor cortex. Here, CLN3 is linked to juvenile neuronal ceroid lipofuscinosis.