Progranulin, lysosomal regulation and neurodegenerative disease

AW Kao, A McKay, PP Singh, A Brunet… - Nature Reviews …, 2017 - nature.com
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …

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 …

Secretory leukocyte protease inhibitor protein regulates the penetrance of frontotemporal lobar degeneration in progranulin mutation carriers

R Ghidoni, R Flocco, A Paterlini… - Journal of …, 2013 - journals.sagepub.com
The discovery that mutations in the gene encoding for progranulin (GRN) cause
frontotemporal lobar degeneration (FTLD) and other neurodegenerative diseases leading to …

Progranulin as a therapeutic target in neurodegenerative diseases

H Rhinn, N Tatton, S McCaughey, M Kurnellas… - Trends in …, 2022 - cell.com
Progranulin (PGRN, encoded by the GRN gene) plays a key role in the development,
survival, function, and maintenance of neurons and microglia in the mammalian brain. It …

Progranulin does not bind tumor necrosis factor (TNF) receptors and is not a direct regulator of TNF-dependent signaling or bioactivity in immune or neuronal cells

X Chen, J Chang, Q Deng, J Xu, TA Nguyen… - Journal of …, 2013 - Soc Neuroscience
Progranulin (PGRN) is a secreted glycoprotein expressed in neurons and glia that is
implicated in neuronal survival on the basis that mutations in the GRN gene causing …

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] Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury

LH Martens, J Zhang, SJ Barmada… - The Journal of …, 2012 - Am Soc Clin Investig
Progranulin (PGRN) is a widely expressed secreted protein that is linked to inflammation. In
humans, PGRN haploinsufficiency is a major inherited cause of frontotemporal dementia …

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

AE Arrant, AJ Filiano, AR Patel, MQ Hoffmann… - Neurobiology of …, 2019 - Elsevier
Loss-of-function mutations in progranulin (GRN), most of which cause progranulin
haploinsufficiency, are a major autosomal dominant cause of frontotemporal dementia …

Progranulin functions as a cathepsin D chaperone to stimulate axonal outgrowth in vivo

S Beel, M Moisse, M Damme… - Human molecular …, 2017 - academic.oup.com
Loss of function mutations in progranulin (GRN) cause frontotemporal dementia, but how
GRN haploinsufficiency causes neuronal dysfunction remains unclear. We previously …

Selective depletion of microglial progranulin in mice is not sufficient to cause neuronal ceroid lipofuscinosis or neuroinflammation

TL Petkau, N Kosior, K de Asis, C Connolly… - Journal of …, 2017 - Springer
Background Progranulin deficiency due to heterozygous null mutations in the GRN gene are
a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous …