Progranulin, lysosomal regulation and neurodegenerative disease

AW Kao, A McKay, PP Singh, A Brunet… - Nature Reviews …, 2017 - nature.com
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …

Progranulin protects against amyloid β deposition and toxicity in Alzheimer's disease mouse models

SS Minami, SW Min, G Krabbe, C Wang, Y Zhou… - Nature medicine, 2014 - nature.com
Haploinsufficiency of the progranulin (PGRN) gene (GRN) causes familial frontotemporal
lobar degeneration (FTLD) and modulates an innate immune response in humans and in …

MicroRNA-29b regulates the expression level of human progranulin, a secreted glycoprotein implicated in frontotemporal dementia

J Jiao, LD Herl, RV Farese Jr, FB Gao - PloS one, 2010 - journals.plos.org
Progranulin deficiency is thought to cause some forms of frontotemporal dementia (FTD), a
major early-onset age-dependent neurodegenerative disease. How progranulin (PGRN) …

CSF progranulin increases in the course of Alzheimer's disease and is associated with sTREM 2, neurodegeneration and cognitive decline

M Suárez‐Calvet, A Capell… - EMBO molecular …, 2018 - embopress.org
Progranulin (PGRN) is predominantly expressed by microglia in the brain, and genetic and
experimental evidence suggests a critical role in Alzheimer's disease (AD). We asked …

Progranulin deficiency causes impairment of autophagy and TDP-43 accumulation

MC Chang, K Srinivasan, BA Friedman… - Journal of Experimental …, 2017 - rupress.org
Loss-of-function mutations in GRN cause frontotemporal dementia (FTD) with transactive
response DNA-binding protein of 43 kD (TDP-43)–positive inclusions and neuronal ceroid …

Progranulin is expressed within motor neurons and promotes neuronal cell survival

CL Ryan, DC Baranowski, BP Chitramuthu, S Malik… - BMC neuroscience, 2009 - Springer
Background Progranulin is a secreted high molecular weight growth factor bearing seven
and one half copies of the cysteine-rich granulin-epithelin motif. While inappropriate over …

Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members

NC Finch, M Baker, R Crook, K Swanson, K Kuntz… - Brain, 2009 - academic.oup.com
Mutations in the progranulin gene (GRN) are an important cause of frontotemporal lobar
degeneration (FTLD) with ubiquitin and TAR DNA-binding protein 43 (TDP43)-positive …

Progranulin gene therapy improves lysosomal dysfunction and microglial pathology associated with frontotemporal dementia and neuronal ceroid lipofuscinosis

AE Arrant, VC Onyilo, DE Unger… - Journal of …, 2018 - Soc Neuroscience
Loss-of-function mutations in progranulin, a lysosomal glycoprotein, cause
neurodegenerative disease. Progranulin haploinsufficiency causes frontotemporal dementia …

The neurotrophic properties of progranulin depend on the granulin E domain but do not require sortilin binding

L De Muynck, S Herdewyn, S Beel, W Scheveneels… - Neurobiology of …, 2013 - Elsevier
Progranulin (PGRN) is a growth factor involved in wound healing, inflammation, tumor
growth, and neurodegeneration. Mutations in the gene encoding PGRN give rise to shortage …

Regulation of progranulin expression in human microglia and proteolysis of progranulin by matrix metalloproteinase-12 (MMP-12)

HS Suh, N Choi, L Tarassishin, SC Lee - PloS one, 2012 - journals.plos.org
Background The essential role of progranulin (PGRN) as a neurotrophic factor has been
demonstrated by the discovery that haploinsufficiency due to GRN gene mutations causes …