Progranulin: a new avenue towards the understanding and treatment of neurodegenerative disease

BP Chitramuthu, HPJ Bennett, A Bateman - Brain, 2017 - academic.oup.com
Progranulin, a secreted glycoprotein, is encoded in humans by the single GRN gene.
Progranulin consists of seven and a half, tandemly repeated, non-identical copies of the 12 …

[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 …

Using the tube test to measure social hierarchy in mice

Z Fan, H Zhu, T Zhou, S Wang, Y Wu, H Hu - Nature Protocols, 2019 - nature.com
Investigation of the neural mechanisms underlying social hierarchy requires a reliable and
effective behavioral test. The tube test is a simple and robust behavioral assay that we …

Latozinemab, a novel progranulin-elevating therapy for frontotemporal dementia

M Kurnellas, A Mitra, T Schwabe, R Paul… - Journal of Translational …, 2023 - Springer
Background Heterozygous loss-of-function mutations in the progranulin (PGRN) gene
(GRN) cause a reduction in PGRN and lead to the development of frontotemporal dementia …

Behavioral defects associated with amygdala and cortical dysfunction in mice with seeded α-synuclein inclusions

LE Stoyka, AE Arrant, DR Thrasher, DL Russell… - Neurobiology of …, 2020 - Elsevier
Parkinson's disease (PD) is defined by motor symptoms such as tremor at rest, bradykinesia,
postural instability, and stiffness. In addition to the classical motor defects that define PD, up …

Restoring neuronal progranulin reverses deficits in a mouse model of frontotemporal dementia

AE Arrant, AJ Filiano, DE Unger, AH Young… - Brain, 2017 - academic.oup.com
Loss-of-function mutations in progranulin (GRN), a secreted glycoprotein expressed by
neurons and microglia, are a common autosomal dominant cause of frontotemporal …

Loss of Tmem106b exacerbates FTLD pathologies and causes motor deficits in progranulin‐deficient mice

X Zhou, M Brooks, P Jiang, S Koga, AR Zuberi… - EMBO …, 2020 - embopress.org
Progranulin (PGRN) and transmembrane protein 106B (TMEM 106B) are important
lysosomal proteins implicated in frontotemporal lobar degeneration (FTLD) and other …

Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay

AD Nguyen, TA Nguyen, J Zhang… - Proceedings of the …, 2018 - National Acad Sciences
Frontotemporal dementia (FTD) is the most common neurodegenerative disorder in
individuals under age 60 and has no treatment or cure. Because many cases of FTD result …

Neural circuit and synaptic dysfunctions in ALS-FTD pathology

S Mora, I Allodi - Frontiers in Neural Circuits, 2023 - frontiersin.org
Action selection is a capital feature of cognition that guides behavior in processes that range
from motor patterns to executive functions. Here, the ongoing actions need to be monitored …

A novel pathway regulates social hierarchy via lncRNA AtLAS and postsynaptic synapsin IIb

M Ma, W Xiong, F Hu, MF Deng, X Huang, JG Chen… - Cell research, 2020 - nature.com
Dominance hierarchy is a fundamental phenomenon in grouped animals and human
beings, however, the underlying regulatory mechanisms remain elusive. Here, we report that …