CLN1 disease is a fatal pediatric neurodegenerative disorder caused by autosomal recessive loss-of function mutations in the PPT1 gene. PPT1 encodes lysosomal enzyme palmitoyl protein thioesterase-1 (PPT1). PPT1 deficiency causes build-up of storage material in many cell populations, but for unknown reasons leads to particular devastation in the CNS. Previous therapeutic approaches in Ppt1-/- mice used AAV-mediated gene therapy to the brain and spinal cord to replace the missing enzyme. While this strategy significantly improved survival, Ppt1-/- mice still died prematurely. New preliminary data suggest CLN1 disease also causes profound pathological changes in the enteric nervous system (ENS) and bowel motility abnormalities. Gut dysfunction is also common in children with CLN1 disease. Here we characterize and measure ex vivo neurogenic motility patterns in the colon and distal small bowel of Ppt1-/- mice at an advanced disease stage using an organ bath apparatus.