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Induced Pluripotent Stem Cells Derived from a CLN5 Patient Manifest Phenotypic Characteristics of Neuronal Ceroid Lipofuscinoses
Neuronal ceroid lipofuscinoses (NCLs) are autosomal recessive progressive encephalopathies caused by mutations in at least 14 different genes. Despite extensive studies performed in different NCL animal models, the molecular mechanisms underlying neurodegeneration in NCLs remain poorly understood. T...
Autores principales: | Uusi-Rauva, Kristiina, Blom, Tea, von Schantz-Fant, Carina, Blom, Tomas, Jalanko, Anu, Kyttälä, Aija |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5454868/ https://www.ncbi.nlm.nih.gov/pubmed/28468312 http://dx.doi.org/10.3390/ijms18050955 |
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