Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3Δex7/8-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

Developmental impairments of select neur

Developmental impairments of select neurotransmitter systems in brains of Cln3Δex7/8 knock-in mice, an animal model of juvenile neuronal ceroid lipofuscinosis

✍ Philipp Herrmann; Caroline Druckrey-Fiskaaen; Elena Kouznetsova; Katrin Heinitz; 📂 Article 📅 2008 🏛 John Wiley and Sons 🌐 English ⚖ 691 KB

## Abstract The neuronal ceroidlipofuscinoses (NCL) are a group of neurodegenerative disorders and are the most common lysosomal storage diseases of infancy and childhood. Juvenile NCL is caused by __CLN3__ mutation, producing retinal degeneration, uncontrollable seizures, cognitive and motor decli