Potential mechanisms of progranulin-deficient FTLD

ME Ward, BL Miller - Journal of Molecular Neuroscience, 2011 - Springer
Frontotemporal lobar dementia (FTLD) is the most common cause of dementia in patients
younger than 60 years of age, and causes progressive neurodegeneration of the frontal and …

Core features of frontotemporal dementia recapitulated in progranulin knockout mice

N Ghoshal, JT Dearborn, DF Wozniak, NJ Cairns - Neurobiology of disease, 2012 - Elsevier
Frontotemporal dementia (FTD) is typified by behavioral and cognitive changes manifested
as altered social comportment and impaired memory performance. To investigate the …

Impaired β-glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations

AE Arrant, JR Roth, NR Boyle, SN Kashyap… - Acta neuropathologica …, 2019 - Springer
Loss-of-function mutations in progranulin (GRN) are a major autosomal dominant cause of
frontotemporal dementia. Most pathogenic GRN mutations result in progranulin …

[HTML][HTML] Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

F Yin, M Dumont, R Banerjee, Y Ma, H Li… - The FASEB …, 2010 - ncbi.nlm.nih.gov
Progranulin haploinsufficiency causes frontotemporal dementia with tau-negative, ubiquitin-
positive neuronal inclusion pathology. In this study, we showed that progranulin-deficient …

Progranulin in neurodegenerative disease

TL Petkau, BR Leavitt - Trends in neurosciences, 2014 - cell.com
Loss-of-function mutations in the progranulin gene are a common cause of familial
frontotemporal dementia (FTD). The purpose of this review is to summarize the role of …

Progranulin: functions and neurologic correlations

RA Townley, BF Boeve, EE Benarroch - Neurology, 2018 - AAN Enterprises
Progranulin is a highly conserved secreted protein that is expressed in multiple cell types,
both in the CNS and in peripheral tissues. Both directly and via its conversion to granulins …

Progranulin axis and recent developments in frontotemporal lobar degeneration

AM Nicholson, J Gass, L Petrucelli… - Alzheimer's Research & …, 2012 - Springer
Frontotemporal lobar degeneration (FTLD) is a devastating neurodegenerative disease that
is the second most common form of dementia affecting individuals under age 65. The most …

[HTML][HTML] Regulation of extracellular progranulin in medial prefrontal cortex

AK Kaplelach, SN Fox, AK Cook, JA Hall… - Neurobiology of …, 2023 - Elsevier
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 …

Delivering progranulin to neuronal lysosomes protects against excitotoxicity

SE Davis, JR Roth, Q Aljabi, AR Hakim… - Journal of Biological …, 2021 - ASBMB
Loss-of-function mutations in progranulin (GRN) are a major genetic cause of frontotemporal
dementia (FTD), possibly due to loss of progranulin's neurotrophic and anti-inflammatory …

Genetic regulation of neuronal progranulin reveals a critical role for the autophagy-lysosome pathway

LP Elia, AR Mason, A Alijagic… - Journal of …, 2019 - Soc Neuroscience
Deficient progranulin levels cause dose-dependent neurological syndromes:
haploinsufficiency leads to frontotemporal lobar degeneration (FTLD) and nullizygosity …