The progranulin cleavage product granulin 3 exerts a dominant negative effect on animal fitness

AL Wang, EA Mambou, AW Kao - Human Molecular Genetics, 2024 - academic.oup.com
Progranulin is an evolutionarily conserved protein that has been implicated in human
neurodevelopmental and neurodegenerative diseases. Human progranulin is comprised of …

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 …

The progranulin cleavage products, granulins, exacerbate TDP-43 toxicity and increase TDP-43 levels

DA Salazar, VJ Butler, AR Argouarch… - Journal of …, 2015 - Soc Neuroscience
Mutations in the human progranulin gene resulting in protein haploinsufficiency cause
frontotemporal lobar degeneration with TDP-43 inclusions. Although progress has been …

Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration

B Cenik, CF Sephton, BK Cenik, J Herz, G Yu - Journal of Biological …, 2012 - ASBMB
GRN mutations cause frontotemporal lobar degeneration with TDP-43-positive inclusions.
The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN …

Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration

S Mohan, PJ Sampognaro, AR Argouarch… - Molecular …, 2021 - Springer
Background Progranulin loss-of-function mutations are linked to frontotemporal lobar
degeneration with TDP-43 positive inclusions (FTLD-TDP-Pgrn). Progranulin (PGRN) is an …

Progranulin regulates neuronal outgrowth independent of sortilin

J Gass, WC Lee, C Cook, N Finch, C Stetler… - Molecular …, 2012 - Springer
Background Progranulin (PGRN), a widely secreted growth factor, is involved in multiple
biological functions, and mutations located within the PGRN gene (GRN) are a major cause …

Progranulin and TDP-43: mechanistic links and future directions

S Kumar-Singh - Journal of molecular neuroscience, 2011 - Springer
Loss-of-function mutations in the multifunctional growth factor progranulin (GRN) cause
frontotemporal lobar degeneration (FTLD) with TDP-43 protein accumulation. Nuclear TDP …

Differential regulation of progranulin derived granulin peptides

T Zhang, H Du, MN Santos, X Wu, MD Pagan… - Molecular …, 2022 - Springer
Background Haploinsufficiency of progranulin (PGRN) is a leading cause of frontotemporal
lobar degeneration (FTLD). PGRN is comprised of 7.5 granulin repeats and is processed …

[HTML][HTML] Human progranulin-expressing mice as a novel tool for the development of progranulin-modulating therapeutics

TL Petkau, B Life, G Lu, J Yang, O Fornes… - Neurobiology of …, 2021 - Elsevier
The granulin protein (also known as, and hereafter referred to as, progranulin) is a secreted
glycoprotein that contributes to overall brain health. Heterozygous loss-of-function mutations …

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 …