Frontotemporal dementia patient neurons with progranulin deficiency display protein dyshomeostasis

L Elia, B Herting, A Alijagic, C Buselli, L Wong… - bioRxiv, 2023 - biorxiv.org
Haploinsufficiency of progranulin (PGRN) causes frontotemporal dementia (FTD), a
devastating neurodegenerative disease with no effective treatment. PGRN is required for …

[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 …

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 …

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 …

[PDF][PDF] 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 …

Dissociation of frontotemporal dementia–related deficits and neuroinflammation in progranulin haploinsufficient mice

AJ Filiano, LH Martens, AH Young… - Journal of …, 2013 - Soc Neuroscience
Frontotemporal dementia (FTD) is a neurodegenerative disease with hallmark deficits in
social and emotional function. Heterozygous loss-of-function mutations in GRN, the …

Lipid Dysregulation Unveil the Intricate Interplay of Lysosomal and Mitochondrial Changes in Frontotemporal Dementia with GRN Haploinsufficiency

J Ondaro, JL Zuniga-Elizari, M Zufiria… - bioRxiv, 2024 - biorxiv.org
This study investigates the cellular pathology resulting from haploinsufficiency of progranulin
(PGRN) in frontotemporal dementia (FTD) associated with granulin (GRN) mutations …

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 …

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 …

Overexpression of progranulin increases pathological protein accumulation by suppressing autophagic flux

Y Tanaka, S Kusumoto, Y Honma, K Takeya… - … and Biophysical Research …, 2022 - Elsevier
Progranulin (PGRN) haploinsufficiency from autosomal dominant mutations in the PGRN
gene causes frontotemporal lobar degeneration, which is characterized by cytoplasmic …