[HTML][HTML] Granulin in frontotemporal lobar degeneration: molecular mechanisms of the disease

ZN Karamysheva, EB Tikhonova… - Frontiers in …, 2019 - frontiersin.org
Frontotemporal lobar degeneration (FTLD) is a pathological process characterized by
severe atrophy in the frontal and temporal lobes of the brain (Mackenzie et al., 2011). There …

[HTML][HTML] Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat frontotemporal …

CJ Holler, G Taylor, ZT McEachin, Q Deng… - Molecular …, 2016 - Springer
Background Progranulin (PGRN) is a secreted growth factor important for neuronal survival
and may do so, in part, by regulating lysosome homeostasis. Mutations in the PGRN gene …

Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration

J Gass, A Cannon, IR Mackenzie… - Human molecular …, 2006 - academic.oup.com
Null mutations in the progranulin gene (PGRN) were recently reported to cause tau-negative
frontotemporal dementia linked to chromosome 17. We assessed the genetic contribution of …

Losing protein in the brain: the case of progranulin

R Ghidoni, A Paterlini, V Albertini, G Binetti, L Benussi - Brain research, 2012 - Elsevier
It is well known that progranulin protein is involved in wound repair, inflammation, and tumor
formation. The wedding between progranulin and brain was celebrated in 2006 with the …

Progranulin mutations in clinical and neuropathological Alzheimer's disease

BN Vardarajan, D Reyes‐Dumeyer… - Alzheimer's & …, 2022 - Wiley Online Library
Introduction Progranulin (GRN) mutations occur in frontotemporal lobar degeneration
(FTLD) and in Alzheimer's disease (AD), often with TDP‐43 pathology. Methods We …

[HTML][HTML] Lysosomal functions of progranulin and implications for treatment of frontotemporal dementia

MJ Simon, T Logan, SL DeVos, G Di Paolo - Trends in Cell Biology, 2023 - cell.com
Loss-of-function heterozygous mutations in GRN, the gene encoding progranulin (PGRN),
were identified in patients with frontotemporal lobar degeneration (FTLD) almost two …

[HTML][HTML] Promoter DNA methylation regulates progranulin expression and is altered in FTLD

J Banzhaf-Strathmann, R Claus, O Mücke… - Acta neuropathologica …, 2013 - Springer
Background Frontotemporal lobar degeneration (FTLD) is a heterogeneous group of
neurodegenerative diseases associated with personality changes and progressive …

Recent insights into the involvement of progranulin in frontotemporal dementia

L Sun, JL Eriksen - Current neuropharmacology, 2011 - ingentaconnect.com
Progranulin is a widely expressed protein that is involved in the regulation of multiple
biological processes, including embryogenesis, host defense, and wound repair. In the …

[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 …

[HTML][HTML] Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis

S Boland, S Swarup, YA Ambaw, PC Malia… - Nature …, 2022 - nature.com
Haploinsufficiency of GRN causes frontotemporal dementia (FTD). The GRN locus produces
progranulin (PGRN), which is cleaved to lysosomal granulin polypeptides. The function of …