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Increased Expression of the Large Conductance, Calcium-Activated K(+) (BK) Channel in Adult-Onset Neuronal Ceroid Lipofuscinosis

Cysteine string protein (CSPα) is a presynaptic J protein co-chaperone that opposes neurodegeneration. Mutations in CSPα (i.e., Leu115 to Arg substitution or deletion (Δ) of Leu116) cause adult neuronal ceroid lipofuscinosis (ANCL), a dominantly inherited neurodegenerative disease. We have previousl...

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Detalles Bibliográficos
Autores principales: Donnelier, Julien, Braun, Samuel T., Dolzhanskaya, Natalia, Ahrendt, Eva, Braun, Andrew P., Velinov, Milen, Braun, Janice E. A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4407904/
https://www.ncbi.nlm.nih.gov/pubmed/25905915
http://dx.doi.org/10.1371/journal.pone.0125205