Developed in conjunction with the University of Pennsylvania Perelman School of Medicine.
CLN2 disease, a form of Batten disease, is a neurodegenerative lysosomal storage disorder caused by mutations in the gene encoding the soluble enzyme tripeptidyl- peptidase-1 (TPP1). This model recapitulates most features of the human disease such as shortened lifespan, seizures, or abnormal gait. Monitoring of the neurobehavioral function in this model is challenging, however, as they are prone to noise- or stress-induced fatal seizures when handled. Real time non-invasive continuous monitoring using smart cages (Vium, Inc.) allows sensitive and non-biased recording of disease phenotype and treatment-related rescue in a seizure-prone mouse model, while limiting handling-related deaths.