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Astrocytes in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3) display metabolic and calcium signaling abnormalities

Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) is a lysosomal storage disease caused by autosomal recessive mutations in CLN3. Children with JNCL experience progressive visual, cognitive, and motor deterioration with a decreased life expectancy (late teens-early 20s). Neuronal loss is thought to occ...

詳細記述

保存先:
書誌詳細
出版年:J Neurochem
主要な著者: Bosch, Megan E., Kielian, Tammy
フォーマット: Artigo
言語:Inglês
出版事項: 2018
主題:
オンライン・アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC7233413/
https://ncbi.nlm.nih.gov/pubmed/29964296
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/jnc.14545
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