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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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Autores principales: Macauley, Shannon L., Pekny, Milos, Sands, Mark S.
Formato: Artigo
Lenguaje:Inglês
Publicado: Society for Neuroscience 2011
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Acceso en línea: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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