Results 1 to 10 of about 7,247 (208)

Selective neuronal restoration of progranulin does not prevent the frontotemporal dementia like-phenotype of progranulin knockout mice [PDF]

Journal of Neuroinflammation
Progranulin (PGRN) is a neurotrophic and anti-inflammatory factor produced mainly by neurons and microglia in the central nervous system. Progranulin haploinsufficiency causes frontotemporal dementia (FTD).
Marc-Philipp Weyer, Lisa Hahnefeld, Luisa Franck, Carlo Angioni, Matthias Klein, Gerd Geisslinger, Michael K.E. Schäfer, Irmgard Tegeder   +7 more
doaj   +3 more sources

Regulation of extracellular progranulin in medial prefrontal cortex

Neurobiology of Disease, 2023
Progranulin is a secreted pro-protein that has anti-inflammatory and neurotrophic effects and is necessary for maintaining lysosomal function. Mutations in progranulin (GRN) are a major cause of frontotemporal dementia.
Andrew E Arrant
exaly   +3 more sources

Analysing the effect of full-length and C-terminally truncated progranulin on proliferation, colony formation, and migration in HepG2 and U87 cells [PDF]

Scientific Reports
Progranulin, the precursor protein to seven and a half distinct granulin motifs (GRNs), has been implicated in a broad range of diseases. Progranulin depletion is one of the most frequent causes for hereditary Frontotemporal Dementia (FTD).
Alexander M. Hofer, Luisa Tobler, Marc-David Ruepp, Oliver Mühlemann   +3 more
doaj   +3 more sources

Serum levels of PTEN and progranulin as potential diagnostic and prognostic biomarkers for severe pneumonia in the elderly [PDF]

PeerJ
Background Severe pneumonia presents a critical challenge clinically, especially for elderly patients. This study investigates the diagnostic and prognostic potential of serum Phosphatase and Tensin Homolog Deleted on Chromosome Ten (PTEN) and ...
Yi Ge, Wei Lu, Jinlong Liu, Huan Liang, Linlin Xu   +4 more
doaj   +3 more sources

FTD-associated behavioural and transcriptomic abnormalities in ‘humanized’ progranulin-deficient mice: A novel model for progranulin-associated FTD

Neurobiology of Disease, 2023
Frontotemporal dementia (FTD) is an early onset dementia characterized by neuropathology and behavioural changes. A common genetic cause of FTD is haploinsufficiency of the gene progranulin (GRN).
Terri L Petkau, Erick I Navarro-Delgado, Keegan Korthäuer   +2 more
exaly   +3 more sources

Reduction of microglial progranulin does not exacerbate pathology or behavioral deficits in neuronal progranulin-insufficient mice

Neurobiology of Disease, 2019
Loss-of-function mutations in progranulin (GRN), most of which cause progranulin haploinsufficiency, are a major autosomal dominant cause of frontotemporal dementia (FTD).
Andrew E Arrant, Anthony J Filiano, Erik D Robérson   +2 more
exaly   +3 more sources

Reduction of sphingomyelinase activity associated with progranulin deficiency and frontotemporal dementia [PDF]

Neurobiology of Disease
Loss-of-function mutations affecting the lysosomal protein progranulin are a leading cause of frontotemporal dementia. Progranulin mutations cause abnormalities in lysosomal lipid processing, particularly of sphingolipids, major components of neural cell
Nicholas R. Boyle, Stephanie N. Fox, Aniketh S. Tadepalli, Nicholas T. Seyfried, Thomas Kukar, Eliana M. Ramos, Alissa L. Nana, Salvatore Spina, Lea T. Grinberg, Bruce L. Miller, William W. Seeley, Andrew E. Arrant, Erik D. Roberson   +12 more
doaj   +2 more sources

Phenotypic diversity of frontotemporal lobar degeneration in two novel GRN variants from Colombia. [PDF]

Alzheimers Dement
Abstract INTRODUCTION Pathogenic progranulin (GRN) variants are among the main genetic causes of frontotemporal lobar degeneration (FTLD). These variants have been predominantly reported in European cohorts, but their characterization in Latin America remains scarce.
Barbosa-Carvajal JP, García-García M, Gómez Navarro LF, Zubiri V, Gelvez N, López G, Reyes P, García-Cifuentes E, Aguillón D, Acosta-Uribe J, Matallana DL.   +10 more
europepmc   +2 more sources

Intrathalamic delivery of adeno-associated viral vector expressing progranulin as gene therapy for GRN-related frontotemporal dementia [PDF]

Molecular Therapy: Methods & Clinical Development
GRN mutations leading to progranulin haploinsufficiency can cause frontotemporal dementia. AVB-101, an investigational gene therapy comprising an adeno-associated virus construct expressing codon-optimized human GRN under a neuronal promoter, was ...
Youn Bok Lee, Do Young Lee, Zoe Walker, Joseph Allison, Aleksandra Kaliszewska, Alinda Fernandes, Katharine Y. Zhang, Yalem Bekele, Paniz Hosseini, Romain Joubert, Joana Furtado, McQuinn Gumus, Olivier Brock, Ali Bienemann, Steven Gill, Samantha Murray, Nadia Mitchell, Page Bouchard, Alex Bloom, John Isaac, Carlos J. Miranda, Christopher E. Shaw   +21 more
doaj   +2 more sources

Synaptic dysfunction in progranulin-deficient mice

Neurobiology of Disease, 2012
Progranulin haploinsufficiency is a common cause of familial frontotemporal dementia (FTD), but the role of progranulin in the brain is poorly understood.
Terri L Petkau, Scott J Neal, Howard H Feldman   +2 more
exaly   +3 more sources