[HTML][HTML] Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations

J Kocerha, N Kouri, M Baker, NC Finch… - BMC genomics, 2011 - Springer
Background Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative
disorder that can be triggered through genetic or sporadic mechanisms. MicroRNAs …

Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures

A Guo, L Tapia, SX Bamji, MS Cynader, W Jia - Brain research, 2010 - Elsevier
Null mutations in the progranulin gene (PGRN) have been identified as a major cause of
frontotemporal dementia with ubiquitinated inclusions. In this disorder, ubiquitinated …

Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia

R Rademakers, JL Eriksen, M Baker… - Human molecular …, 2008 - academic.oup.com
Loss-of-function mutations in progranulin (GRN) cause ubiquitin-and TAR DNA-binding
protein 43 (TDP-43)-positive frontotemporal dementia (FTLD-U), a progressive …

[HTML][HTML] Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic

T Logan, MJ Simon, A Rana, GM Cherf, A Srivastava… - Cell, 2021 - cell.com
GRN mutations cause frontotemporal dementia (GRN-FTD) due to deficiency in progranulin
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …

Progranulin haploinsufficiency reduces amyloid beta deposition in Alzheimer's disease model mice

M Hosokawa, Y Tanaka, T Arai, H Kondo… - Experimental …, 2018 - jstage.jst.go.jp
Granulin (Grn) mutations were identified in familial frontotemporal lobar degeneration
(FTLD) patients with TAR DNA-binding protein of 43 kd (TDP-43) pathology. Grn transcript …

Progranulin: an emerging target for FTLD therapies

J Gass, M Prudencio, C Stetler, L Petrucelli - Brain research, 2012 - Elsevier
Frontotemporal lobar degeneration (FTLD), a neurodegenerative disease primarily affecting
the frontal and temporal lobes, is one of the most common types of dementia. While the …

[HTML][HTML] Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin

F Hu, T Padukkavidana, CB Vægter, OA Brady… - Neuron, 2010 - cell.com
The most common inherited form of Frontotemporal Lobar Degeneration (FTLD) known
stems from Progranulin (GRN) mutation and exhibits TDP-43 plus ubiquitin aggregates …

Rescue of progranulin deficiency associated with frontotemporal lobar degeneration by alkalizing reagents and inhibition of vacuolar ATPase

A Capell, S Liebscher, K Fellerer… - Journal of …, 2011 - Soc Neuroscience
Numerous loss-of-function mutations in the progranulin (GRN) gene cause frontotemporal
lobar degeneration with ubiquitin and TAR–DNA binding protein 43-positive inclusions by …

Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice

TL Petkau, J Blanco, BR Leavitt - Neurobiology of Disease, 2017 - Elsevier
Progranulin deficiency due to heterozygous null mutations in the GRN gene is a common
cause of familial frontotemporal lobar degeneration (FTLD), while homozygous loss-of …

Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

F Yin, R Banerjee, B Thomas, P Zhou, L Qian… - Journal of Experimental …, 2010 - rupress.org
Progranulin (PGRN) is a widely expressed protein involved in diverse biological processes.
Haploinsufficiency of PGRN in the human causes tau-negative, ubiquitin-positive …