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Mutant PrP Suppresses Glutamatergic Neurotransmission in Cerebellar Granule Neurons by Impairing Membrane Delivery of VGCC α(2)δ-1 Subunit
How mutant prion protein (PrP) leads to neurological dysfunction in genetic prion diseases is unknown. Tg(PG14) mice synthesize a misfolded mutant PrP which is partially retained in the neuronal endoplasmic reticulum (ER). As these mice age, they develop ataxia and massive degeneration of cerebellar...
Autores principales: | Senatore, Assunta, Colleoni, Simona, Verderio, Claudia, Restelli, Elena, Morini, Raffaella, Condliffe, Steven B., Bertani, Ilaria, Mantovani, Susanna, Canovi, Mara, Micotti, Edoardo, Forloni, Gianluigi, Dolphin, Annette C., Matteoli, Michela, Gobbi, Marco, Chiesa, Roberto |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cell Press
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3339322/ https://www.ncbi.nlm.nih.gov/pubmed/22542184 http://dx.doi.org/10.1016/j.neuron.2012.02.027 |
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