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Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

BACKGROUND: Heterozygous loss-of-function mutations in the progranulin gene (GRN) lead to frontotemporal lobar degeneration (FTLD) while the complete loss of progranulin (PGRN) function results in neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disease. Thus the growth factor-like protein...

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Detalles Bibliográficos
Autores principales: Götzl, Julia K., Colombo, Alessio-Vittorio, Fellerer, Katrin, Reifschneider, Anika, Werner, Georg, Tahirovic, Sabina, Haass, Christian, Capell, Anja
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6123925/
https://www.ncbi.nlm.nih.gov/pubmed/30180904
http://dx.doi.org/10.1186/s13024-018-0281-5