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The consequences of CLN3 mutation on the progression of the disease, on neuronal transmission, and on central nervous network dysfunction are poorly understood. We used Cln3 knockout (Cln3\u0394ex1-6) mice and found increased anxiety-related behavior and impaired aversive learning as well as markedly affected motor function including disordered coordination. Patch-clamp and loose-patch recordings revealed severely affected inhibitory and excitatory synaptic transmission in the amygdala, hippocampus, and cerebellar networks. Changes in presynaptic release properties may result from dysfunction of CLN3 protein. Furthermore, loss of calbindin, neuropeptide Y, parvalbumin, and GAD65-positive interneurons in central networks collectively support the hypothesis that degeneration of GABAergic interneurons may be the cause of supraspinal GABAergic disinhibition.<\/jats:p>","DOI":"10.7554\/elife.28685","type":"journal-article","created":{"date-parts":[[2017,11,14]],"date-time":"2017-11-14T08:00:25Z","timestamp":1510646425000},"source":"Crossref","is-referenced-by-count":39,"title":["Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis"],"prefix":"10.7554","volume":"6","author":[{"given":"Benedikt","family":"Gr\u00fcnewald","sequence":"first","affiliation":[{"name":"Hans-Berger Department of Neurology, Jena University Hospital, Jena, Germany"},{"name":"Integrated Research and Treatment Center\u2014Center for Sepsis Control and Care, Jena University Hospital, Jena, Germany"},{"name":"Department of Neurology, University Hospital W\u00fcrzburg, 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