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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Bibliographic Details
Main Authors: Burkovetskaya, Maria, Karpuk, Nikolay, Xiong, Juan, Bosch, Megan, Boska, Michael D., Takeuchi, Hideyuki, Suzumura, Akio, Kielian, Tammy
Format: Artigo
Language:Inglês
Published: Public Library of Science 2014
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Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3988164/
https://ncbi.nlm.nih.gov/pubmed/24736558
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0095023
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