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Comprehensive functional characterization of murine infantile Batten disease including Parkinson-like behavior and dopaminergic markers

Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten disease) is a neurodegenerative lysosomal storage disease caused by a deficiency in palmitoyl protein thioesterase-1 (PPT1). The PPT1-deficient mouse (Cln1(−/−)) is a useful phenocopy of human INCL. Cln1(−/−) mice display retinal dysfu...

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Detalles Bibliográficos
Autores principales: Dearborn, Joshua T., Harmon, Steven K., Fowler, Stephen C., O’Malley, Karen L., Taylor, George T., Sands, Mark S., Wozniak, David F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4523849/
https://www.ncbi.nlm.nih.gov/pubmed/26238334
http://dx.doi.org/10.1038/srep12752