[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 …
were identified in patients with frontotemporal lobar degeneration (FTLD) almost two …
Progranulin as a therapeutic target in neurodegenerative diseases
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 …
survival, function, and maintenance of neurons and microglia in the mammalian brain. It …
[HTML][HTML] 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 …
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …
Progranulin deficiency promotes circuit-specific synaptic pruning by microglia via complement activation
H Lui, J Zhang, SR Makinson, MK Cahill, KW Kelley… - Cell, 2016 - cell.com
Microglia maintain homeostasis in the brain, but whether aberrant microglial activation can
cause neurodegeneration remains controversial. Here, we use transcriptome profiling to …
cause neurodegeneration remains controversial. Here, we use transcriptome profiling to …
[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …
neurodegenerative disorders that are thought to exist on a clinical and pathological …
Disorders of lysosomal acidification—The emerging role of v-ATPase in aging and neurodegenerative disease
DJ Colacurcio, RA Nixon - Ageing research reviews, 2016 - Elsevier
Autophagy and endocytosis deliver unneeded cellular materials to lysosomes for
degradation. Beyond processing cellular waste, lysosomes release metabolites and ions …
degradation. Beyond processing cellular waste, lysosomes release metabolites and ions …
Progranulin, lysosomal regulation and neurodegenerative disease
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …
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 …
Loss of TREM2 rescues hyperactivation of microglia, but not lysosomal deficits and neurotoxicity in models of progranulin deficiency
A Reifschneider, S Robinson, B van Lengerich… - The EMBO …, 2022 - embopress.org
Haploinsufficiency of the progranulin (PGRN)‐encoding gene (GRN) causes frontotemporal
lobar degeneration (GRN‐FTLD) and results in microglial hyperactivation, TREM2 …
lobar degeneration (GRN‐FTLD) and results in microglial hyperactivation, TREM2 …
ER stress and impaired autophagy flux in neuronal degeneration and brain injury
Y Yin, G Sun, E Li, K Kiselyov, D Sun - Ageing research reviews, 2017 - Elsevier
Autophagy is a highly controlled lysosome-mediated function in eukaryotic cells to eliminate
damaged or aged long-lived proteins and organelles. It is required for restoring cellular …
damaged or aged long-lived proteins and organelles. It is required for restoring cellular …