Electrophysiological Profile Remodeling via Selective Suppression of Voltage-Gated Currents by CLN1/PPT1 Overexpression in Human Neuronal-Like Cells

CLN1 disease (OMIM #256730) is an inherited neurological disorder of early childhood with epileptic seizures and premature death. It is associated with mutations in CLN1 coding for Palmitoyl-Protein Thioesterase 1 (PPT1), a lysosomal enzyme which affects the recycling and degradation of lipid-modifi...

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Detalles Bibliográficos
Autores principales: Demontis, Gian Carlo, Pezzini, Francesco, Margari, Elisa, Bianchi, Marzia, Longoni, Biancamaria, Doccini, Stefano, Lalowski, Maciej Maurycy, Santorelli, Filippo Maria, Simonati, Alessandro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Cellular Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772423/
https://www.ncbi.nlm.nih.gov/pubmed/33390903
http://dx.doi.org/10.3389/fncel.2020.569598

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772423/
https://www.ncbi.nlm.nih.gov/pubmed/33390903
http://dx.doi.org/10.3389/fncel.2020.569598

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