[HTML][HTML] Lysosomal functions of progranulin and implications for treatment of frontotemporal dementia

MJ Simon, T Logan, SL DeVos, G Di Paolo - Trends in Cell Biology, 2023 - cell.com
Loss-of-function heterozygous mutations in GRN, the gene encoding progranulin (PGRN),
were identified in patients with frontotemporal lobar degeneration (FTLD) almost two …

[HTML][HTML] The lysosomal function of progranulin, a guardian against neurodegeneration

DH Paushter, H Du, T Feng, F Hu - Acta neuropathologica, 2018 - Springer
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 …

Progranulin promotes neurite outgrowth and neuronal differentiation by regulating GSK-3β

X Gao, AP Joselin, L Wang, A Kar, P Ray, A Bateman… - Protein & cell, 2010 - Springer
Progranulin (PGRN) has recently emerged as a key player in a subset of frontotemporal
dementias (FTD). Numerous mutations in the progranulin gene have been identified in …

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 …

Intracellular proteolysis of progranulin generates stable, lysosomal granulins that are haploinsufficient in patients with frontotemporal dementia caused by GRN …

CJ Holler, G Taylor, Q Deng, T Kukar - Eneuro, 2017 - eneuro.org
Homozygous or heterozygous mutations in the GRN gene, encoding progranulin (PGRN),
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …

Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin

X Zhou, L Sun, F Bastos de Oliveira, X Qi… - Journal of Cell …, 2015 - rupress.org
Mutations in the progranulin (PGRN) gene have been linked to two distinct
neurodegenerative diseases, frontotemporal lobar degeneration (FTLD) and neuronal …

[HTML][HTML] Progranulin as a therapeutic target in neurodegenerative diseases

H Rhinn, N Tatton, S McCaughey, M Kurnellas… - Trends in …, 2022 - cell.com
Progranulin (PGRN, encoded by the GRN gene) plays a key role in the development,
survival, function, and maintenance of neurons and microglia in the mammalian brain. It …

[HTML][HTML] Progranulin deficiency leads to reduced glucocerebrosidase activity

X Zhou, DH Paushter, MD Pagan, D Kim… - PLoS …, 2019 - journals.plos.org
Mutation in the GRN gene, encoding the progranulin (PGRN) protein, shows a dose-
dependent disease correlation, wherein haploinsufficiency results in frontotemporal lobar …

[PDF][PDF] Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic

T Logan, MJ Simon, A Rana, GM Cherf, A Srivastava… - Cell, 2021 - cell.com
GRN mutations cause frontotemporal dementia (GRN-FTD) due to deficiency in progranulin
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …

[HTML][HTML] Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations

X Zhou, L Sun, O Bracko, JW Choi, Y Jia… - Nature …, 2017 - nature.com
Haploinsufficiency of progranulin (PGRN) due to mutations in the granulin (GRN) gene
causes frontotemporal lobar degeneration (FTLD), and complete loss of PGRN leads to a …