Progranulin acts as a shared chaperone and regulates multiple lysosomal enzymes
Multifunctional factor progranulin (PGRN) plays an important role in lysosomes, and its
mutations and insufficiency are associated with lysosomal storage diseases, including …
mutations and insufficiency are associated with lysosomal storage diseases, including …
Progranulin regulates lysosomal function and biogenesis through acidification of lysosomes
Y Tanaka, G Suzuki, T Matsuwaki… - Human molecular …, 2017 - academic.oup.com
Progranulin (PGRN) haploinsufficiency resulting from loss-of-function mutations in the
PGRN gene causes frontotemporal lobar degeneration accompanied by TDP-43 …
PGRN gene causes frontotemporal lobar degeneration accompanied by TDP-43 …
Progranulin deficiency leads to reduced glucocerebrosidase activity
Mutation in the GRN gene, encoding the progranulin (PGRN) protein, shows a dose-
dependent disease correlation, wherein haploinsufficiency results in frontotemporal lobar …
dependent disease correlation, wherein haploinsufficiency results in frontotemporal lobar …
The lysosomal function of progranulin, a guardian against neurodegeneration
Progranulin (PGRN), encoded by the GRN gene in humans, is a secreted growth factor
implicated in a multitude of processes ranging from regulation of inflammation to wound …
implicated in a multitude of processes ranging from regulation of inflammation to wound …
Granulins rescue inflammation, lysosome dysfunction, lipofuscin, and neuropathology in a mouse model of progranulin deficiency
J Root, A Mendsaikhan, G Taylor, P Merino, S Nandy… - Cell reports, 2024 - cell.com
Progranulin (PGRN) deficiency is linked to neurodegenerative diseases, including
frontotemporal dementia (FTD), Alzheimer's disease, and Parkinson's disease. Proper …
frontotemporal dementia (FTD), Alzheimer's disease, and Parkinson's disease. Proper …
Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice
JK Götzl, AV Colombo, K Fellerer… - Molecular …, 2018 - Springer
Background Heterozygous loss-of-function mutations in the progranulin gene (GRN) lead to
frontotemporal lobar degeneration (FTLD) while the complete loss of progranulin (PGRN) …
frontotemporal lobar degeneration (FTLD) while the complete loss of progranulin (PGRN) …
Progranulin associates with Rab2 and is involved in autophagosome-lysosome fusion in Gaucher disease
Progranulin (PGRN) is a key regulator of lysosomes, and its deficiency has been linked to
various lysosomal storage diseases (LSDs), including Gaucher disease (GD), one of the …
various lysosomal storage diseases (LSDs), including Gaucher disease (GD), one of the …
Progranulin recruits HSP70 to β-glucocerebrosidase and is therapeutic against Gaucher disease
J Jian, QY Tian, A Hettinghouse, S Zhao, H Liu, J Wei… - …, 2016 - thelancet.com
Gaucher disease (GD), the most common lysosomal storage disease, is caused by
mutations in GBA1 encoding of β-glucocerebrosidase (GCase). Recently it was reported that …
mutations in GBA1 encoding of β-glucocerebrosidase (GCase). Recently it was reported that …
Intracellular proteolysis of progranulin generates stable, lysosomal granulins that are haploinsufficient in patients with frontotemporal dementia caused by GRN …
Homozygous or heterozygous mutations in the GRN gene, encoding progranulin (PGRN),
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …
Differential regulation of progranulin derived granulin peptides
Background Haploinsufficiency of progranulin (PGRN) is a leading cause of frontotemporal
lobar degeneration (FTLD). PGRN is comprised of 7.5 granulin repeats and is processed …
lobar degeneration (FTLD). PGRN is comprised of 7.5 granulin repeats and is processed …