Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL).

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: Maria Burkovetskaya, Nikolay Karpuk, Juan Xiong, Megan Bosch, Michael D Boska, Hideyuki Takeuchi, Akio Suzumura, Tammy Kielian
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3988164?pdf=render

Internet

http://europepmc.org/articles/PMC3988164?pdf=render

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