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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...
Autores principales: | Burkovetskaya, Maria, Karpuk, Nikolay, Xiong, Juan, Bosch, Megan, Boska, Michael D., Takeuchi, Hideyuki, Suzumura, Akio, Kielian, Tammy |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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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 |
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