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 …
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 …
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 …
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 …
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
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 …
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 …
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 …
(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 …
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 …
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 …
Overexpression of progranulin increases pathological protein accumulation by suppressing autophagic flux
Progranulin (PGRN) haploinsufficiency from autosomal dominant mutations in the PGRN
gene causes frontotemporal lobar degeneration, which is characterized by cytoplasmic …
gene causes frontotemporal lobar degeneration, which is characterized by cytoplasmic …