Article

GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice

2024-03-30

Abstract excerpt

GABAergic interneuron deficits have been implicated in the epileptogenesis of multiple neurological diseases. While epileptic seizures are a key clinical hallmark of CLN2 disease, a childhood-onset neurodegenerative lysosomal storage disorder caused by a deficiency of tripeptidyl peptidase 1 (TPP1), the etiology of these seizures remains elusive. Given that Cln2 R207X/R207X mice display fatal spontaneous seizure...

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Literature Corpus work: 63234fb0-731e-5000-b490-3c3b4023e4b9
DOI: 10.1101/2024.03.29.587276

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