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

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

Abstract 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...

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Bibliographic Details
Main Authors: Julia K. Götzl, Alessio-Vittorio Colombo, Katrin Fellerer, Anika Reifschneider, Georg Werner, Sabina Tahirovic, Christian Haass, Anja Capell
Format: Article
Language:English
Published: BMC 2018-09-01
Series:Molecular Neurodegeneration
Subjects:
Frontotemporal lobar degeneration
Microglia
Neurodegeneration
Progranulin
Lysosome
Cathepsin
Online Access:http://link.springer.com/article/10.1186/s13024-018-0281-5

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http://link.springer.com/article/10.1186/s13024-018-0281-5

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