CLN3 and juvenile neuronal ceroid lipofuscinosis: Consistent with the predicted loss of CLN3 normal function as the root cause of this recessively inherited disease, all of the established mouse models display recessive features of JNCL including accumulation of ceroid lipofuscin, brain gliosis, neurological dysfunction and neurodegeneration [8], [20], [21], [22], [23], [24], [25].