[HTML][HTML] Preclinical interventions in mouse models of frontotemporal dementia due to progranulin mutations

SN Kashyap, NR Boyle, ED Roberson - Neurotherapeutics, 2023 - Elsevier
Heterozygous loss-of-function mutations in progranulin (GRN) cause frontotemporal
dementia (FTD), a leading cause of early-onset dementia characterized clinically by …

[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 …

Dysregulation of the progranulin-driven autophagy-lysosomal pathway mediates secretion of the nuclear protein TDP-43

Y Tanaka, S Ito, Y Honma, M Hasegawa… - Journal of Biological …, 2023 - ASBMB
The cytoplasmic accumulation of the nuclear protein transactive response DNA-binding
protein 43 kDa (TDP-43) has been linked to the progression of amyotrophic lateral sclerosis …

CNS-wide repopulation by hematopoietic-derived microglia-like cells corrects progranulin deficiency in mice

P Colella, R Sayana, MV Suarez-Nieto, J Sarno… - Nature …, 2024 - nature.com
Hematopoietic stem cell transplantation can deliver therapeutic proteins to the central
nervous system (CNS) through transplant-derived microglia-like cells. However, current …

Epigenetic modulation through BET bromodomain inhibitors as a novel therapeutic strategy for progranulin-deficient frontotemporal dementia

ZC Rosenthal, DM Fass, NC Payne, A She, D Patnaik… - Scientific reports, 2024 - nature.com
Frontotemporal dementia (FTD) is a debilitating neurodegenerative disorder with currently
no disease-modifying treatment options available. Mutations in GRN are one of the most …

Progranulin and GPNMB: interactions in endo-lysosome function and inflammation in neurodegenerative disease

DA Gillett, RL Wallings, O Uriarte Huarte… - Journal of …, 2023 - Springer
Background Alterations in progranulin (PGRN) expression are associated with multiple
neurodegenerative diseases (NDs), including frontotemporal dementia (FTD), Alzheimer's …

The advance on frontotemporal dementia (FTD)'s neuropathology and molecular genetics

J Wang, B Wang, T Zhou - Mediators of Inflammation, 2022 - Wiley Online Library
The morbidity of frontotemporal dementia (FTD), one of the most prevalent dementias
praccox, is second to Alzheimer disease (AD). It is different with AD that FTD has a rapider …

Biochemical, biomarker, and behavioral characterization of the GrnR493X mouse model of frontotemporal dementia

DM Smith, G Aggarwal, ML Niehoff, SA Jones… - Molecular …, 2024 - Springer
Heterozygous loss-of-function mutations in the progranulin gene (GRN) are a major cause of
frontotemporal dementia due to progranulin haploinsufficiency; complete deficiency of …

[HTML][HTML] hiPSC-derived GRN-deficient astrocytes delay spiking activity of developing neurons

C Lee, J Frew, NL Weilinger, S Wendt, W Cai… - Neurobiology of …, 2023 - Elsevier
Frontotemporal dementia (FTD) refers to a group of neurodegenerative disorders that are
characterized by pathology predominantly localized to the frontal and temporal lobes …

Reduction of Sphingomyelinases Associated With Progranulin Deficiency and Frontotemporal Dementia

NR Boyle - 2023 - search.proquest.com
Frontotemporal dementia (FTD) is a leading cause of early-onset dementia and has a
significant socioeconomic burden due to difficulties in diagnosis and delay to diagnosis. FTD …