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Nat Rev Neurosci. 2017 Jun;18(6):325-333. doi: 10.1038/nrn.2017.36. Epub 2017 Apr 24.

Progranulin, lysosomal regulation and neurodegenerative disease.

Author information

1
Department of Neurology, University of California San Francisco, 675 Nelson Rising Lane, San Francisco, California 94158, USA.
2
Department of Genetics, Stanford University, 300 Pasteur Drive, Stanford, California 94305, USA.
3
Department of Pathology, University of California San Francisco and Pathology Service 113B, San Francisco VA Medical Center, 513 Parnassus Avenue, San Francisco, California 94143, USA.

Abstract

The discovery that heterozygous and homozygous mutations in the gene encoding progranulin are causally linked to frontotemporal dementia and lysosomal storage disease, respectively, reveals previously unrecognized roles of the progranulin protein in regulating lysosome biogenesis and function. Given the importance of lysosomes in cellular homeostasis, it is not surprising that progranulin deficiency has pleiotropic effects on neural circuit development and maintenance, stress response, innate immunity and ageing. This Progress article reviews recent advances in progranulin biology emphasizing its roles in lysosomal function and brain innate immunity, and outlines future avenues of investigation that may lead to new therapeutic approaches for neurodegeneration.

PMID:
28435163
PMCID:
PMC6040832
DOI:
10.1038/nrn.2017.36
[Indexed for MEDLINE]
Free PMC Article

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