Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD

Y Wu, W Shao, TW Todd, J Tong, M Yue, S Koga… - Cell reports, 2021 - cell.com
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 …

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 …

Brain progranulin expression in GRN-associated frontotemporal lobar degeneration

AS Chen-Plotkin, J Xiao, F Geser, M Martinez-Lage… - Acta …, 2010 - Springer
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is characterized by
progressive decline in behavior, executive function, and language. Progranulin (GRN) gene …

[HTML][HTML] New insights and therapeutic opportunities for progranulin-deficient frontotemporal dementia

S Amin, G Carling, L Gan - Current Opinion in Neurobiology, 2022 - Elsevier
Frontotemporal dementia (FTD) is the second most common form of dementia. It affects the
frontal and temporal lobes of the brain and has a highly heterogeneous clinical …

Lysosomal processing of progranulin

X Zhou, DH Paushter, T Feng, L Sun… - Molecular …, 2017 - Springer
Background Mutations resulting in progranulin (PGRN) haploinsufficiency cause
frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP), a …

Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN …

M Huang, E Modeste, E Dammer, P Merino… - Acta neuropathologica …, 2020 - Springer
Heterozygous, loss-of-function mutations in the granulin gene (GRN) encoding progranulin
(PGRN) are a common cause of frontotemporal dementia (FTD). 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 …

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 …

Cellular ageing, increased mortality and FTLD‐TDP‐associated neuropathology in progranulin knockout mice

H Wils, G Kleinberger, S Pereson… - The Journal of …, 2012 - Wiley Online Library
Loss‐of‐function mutations in progranulin (GRN) are associated with frontotemporal lobar
degeneration with intraneuronal ubiquitinated protein accumulations composed primarily of …

Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations

J Kocerha, N Kouri, M Baker, NC Finch… - BMC genomics, 2011 - Springer
Background Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative
disorder that can be triggered through genetic or sporadic mechanisms. MicroRNAs …