Granulins rescue inflammation, lysosome dysfunction, lipofuscin, and neuropathology in a mouse model of progranulin deficiency

J Root, A Mendsaikhan, G Taylor, P Merino, S Nandy… - Cell reports, 2024 - cell.com
Progranulin (PGRN) deficiency is linked to neurodegenerative diseases, including
frontotemporal dementia (FTD), Alzheimer's disease, and Parkinson's disease. Proper …

[HTML][HTML] Granulins rescue inflammation, lysosome dysfunction, and neuropathology in a mouse model of progranulin deficiency

J Root, A Mendsaikhan, S Nandy, G Taylor, M Wang… - bioRxiv, 2023 - ncbi.nlm.nih.gov
Progranulin (PGRN) deficiency is linked to neurodegenerative diseases including
frontotemporal dementia, Alzheimer's disease, Parkinson's disease, and neuronal ceroid …

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 …

A brain penetrant progranulin biotherapeutic rescues lysosomal and inflammatory phenotypes in the brain of GRN knockout mice: Lipids in AD and FTD: From genes …

T Logan, S DeVos, MJ Simon, S Davis… - Alzheimer's & …, 2020 - Wiley Online Library
Background Heterozygous loss of function (LOF) mutations in GRN cause frontotemporal
dementia (FTD), a neurodegenerative disorder associated with lysosomal dysfunction, TDP …

Identification of biological pathways regulated by PGRN and GRN peptide treatments using transcriptome analysis

S Rollinson, K Young… - European Journal of …, 2016 - Wiley Online Library
Mutations in progranulin (PGRN) have been linked to two neurodegenerative disorders,
heterozygote mutations with frontotemporal lobar degeneration (FTLD) and homozygote …

Intracellular proteolysis of progranulin generates stable, lysosomal granulins that are haploinsufficient in patients with frontotemporal dementia caused by GRN …

CJ Holler, G Taylor, Q Deng, T Kukar - Eneuro, 2017 - eneuro.org
Homozygous or heterozygous mutations in the GRN gene, encoding progranulin (PGRN),
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …

A Novel In Vivo‐Active Small Molecule Inducer of Progranulin for the Treatment of Frontotemporal Dementia

JF Blain, AYP Chen, MA Brand, JC Lanter… - Alzheimer's & …, 2022 - Wiley Online Library
Background Heterozygous mutations in GRN that lead to haploinsufficiency of the
progranulin (PGRN) protein cause the fatal neurodegenerative disease frontotemporal …

Selective depletion of microglial progranulin in mice is not sufficient to cause neuronal ceroid lipofuscinosis or neuroinflammation

TL Petkau, N Kosior, K de Asis, C Connolly… - Journal of …, 2017 - Springer
Background Progranulin deficiency due to heterozygous null mutations in the GRN gene are
a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous …

[HTML][HTML] Microglia regulate brain progranulin levels through the endocytosis/lysosomal pathway

T Dong, L Tejwani, Y Jung, H Kokubu, K Luttik… - JCI insight, 2021 - ncbi.nlm.nih.gov
Genetic variants in Granulin (GRN), which encodes the secreted glycoprotein progranulin
(PGRN), are associated with several neurodegenerative diseases, including frontotemporal …

Differential regulation of progranulin derived granulin peptides

T Zhang, H Du, MN Santos, X Wu, MD Pagan… - Molecular …, 2022 - Springer
Background Haploinsufficiency of progranulin (PGRN) is a leading cause of frontotemporal
lobar degeneration (FTLD). PGRN is comprised of 7.5 granulin repeats and is processed …