Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis

Juvenile neuronal ceroid lipofuscinosis (JNCL or Batten disease) caused by mutations in the CLN3 gene is the most prevalent inherited neurodegenerative disease in childhood resulting in widespread central nervous system dysfunction and premature death. The consequences of CLN3 mutation on the progre...

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Detalles Bibliográficos
Autores principales: Grünewald, Benedikt, Lange, Maren D, Werner, Christian, O'Leary, Aet, Weishaupt, Andreas, Popp, Sandy, Pearce, David A, Wiendl, Heinz, Reif, Andreas, Pape, Hans C, Toyka, Klaus V, Sommer, Claudia, Geis, Christian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2017
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5724993/
https://www.ncbi.nlm.nih.gov/pubmed/29135436
http://dx.doi.org/10.7554/eLife.28685

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