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The Role of Attenuated Astrocyte Activation in Infantile Neuronal Ceroid Lipofuscinosis

Infantile neuronal ceroid lipofuscinosis (INCL) is an inherited neurodegenerative disorder affecting the CNS during infancy. INCL is caused by mutations in the CLN1 gene that lead to a deficiency in the lysosomal hydrolase, palmitoyl protein thioesterase 1 (PPT1). A murine model of INCL, the PPT1-de...

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Autori principali: Macauley, Shannon L., Pekny, Milos, Sands, Mark S.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Society for Neuroscience 2011
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3218425/
https://ncbi.nlm.nih.gov/pubmed/22031903
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.3579-11.2011
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