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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...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2015
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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 |