Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN …
Heterozygous, loss-of-function mutations in the granulin gene (GRN) encoding progranulin
(PGRN) are a common cause of frontotemporal dementia (FTD). Homozygous GRN …
(PGRN) are a common cause of frontotemporal dementia (FTD). Homozygous GRN …
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 …
Astroglial toxicity promotes synaptic degeneration in the thalamocortical circuit in frontotemporal dementia with GRN mutations
E Marsan, D Velmeshev, A Ramsey… - The Journal of …, 2023 - Am Soc Clin Investig
Mutations in the human progranulin (GRN) gene are a leading cause of frontotemporal lobar
degeneration (FTLD). While previous studies implicate aberrant microglial activation as a …
degeneration (FTLD). While previous studies implicate aberrant microglial activation as a …
Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons
S Hasan, MS Fernandopulle, SW Humble… - Molecular …, 2023 - Springer
Background Progranulin (PGRN) is a lysosomal glycoprotein implicated in various
neurodegenerative diseases, including frontotemporal dementia and neuronal ceroid …
neurodegenerative diseases, including frontotemporal dementia and neuronal ceroid …
Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD
Loss-of-function mutations in the progranulin gene (GRN), which encodes progranulin
(PGRN), are a major cause of frontotemporal dementia (FTD). GRN-associated FTD is …
(PGRN), are a major cause of frontotemporal dementia (FTD). GRN-associated FTD is …
Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration
S Mohan, PJ Sampognaro, AR Argouarch… - Molecular …, 2021 - Springer
Background Progranulin loss-of-function mutations are linked to frontotemporal lobar
degeneration with TDP-43 positive inclusions (FTLD-TDP-Pgrn). Progranulin (PGRN) is an …
degeneration with TDP-43 positive inclusions (FTLD-TDP-Pgrn). Progranulin (PGRN) is an …
Functional genomic analyses identify pathways dysregulated by progranulin deficiency, implicating Wnt signaling
EY Rosen, EM Wexler, R Versano, G Coppola, F Gao… - Neuron, 2011 - cell.com
Progranulin (GRN) mutations cause frontotemporal dementia (FTD), but GRN's function in
the CNS remains largely unknown. To identify the pathways downstream of GRN, we used …
the CNS remains largely unknown. To identify the pathways downstream of GRN, we used …
Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration
GRN mutations cause frontotemporal lobar degeneration with TDP-43-positive inclusions.
The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN …
The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN …
Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay
AD Nguyen, TA Nguyen, J Zhang… - Proceedings of the …, 2018 - National Acad Sciences
Frontotemporal dementia (FTD) is the most common neurodegenerative disorder in
individuals under age 60 and has no treatment or cure. Because many cases of FTD result …
individuals under age 60 and has no treatment or cure. Because many cases of FTD result …
[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 …
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