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Evidence for Aberrant Astrocyte Hemichannel Activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL)

Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) is a lysosomal storage disease caused by an autosomal recessive mutation in CLN3 that leads to vision loss, progressive cognitive and motor decline, and premature death. Morphological evidence of astrocyte activation occurs early in the disease process...

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Detalles Bibliográficos
Autores principales: Burkovetskaya, Maria, Karpuk, Nikolay, Xiong, Juan, Bosch, Megan, Boska, Michael D., Takeuchi, Hideyuki, Suzumura, Akio, Kielian, Tammy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3988164/
https://www.ncbi.nlm.nih.gov/pubmed/24736558
http://dx.doi.org/10.1371/journal.pone.0095023