Progranulinopathy: A diverse realm of disorders linked to Progranulin imbalances

G Huang, J Jian, CJ Liu - Cytokine & Growth Factor Reviews, 2024 - Elsevier
Progranulin (PGRN), encoded by the GRN gene in humans, was originally isolated as a
secreted growth factor that implicates in a multitude of processes ranging from regulation of …

Autophagy in the neuronal ceroid lipofuscinoses (Batten disease)

WD Kim, MLDM Wilson-Smillie… - Frontiers in Cell and …, 2022 - frontiersin.org
The neuronal ceroid lipofuscinoses (NCLs), also referred to as Batten disease, are a family
of neurodegenerative diseases that affect all age groups and ethnicities around the globe. At …

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

S Hasan, MS Fernandopulle, SW Humble… - Molecular …, 2023 - Springer
Background Progranulin (PGRN) is a lysosomal glycoprotein implicated in various
neurodegenerative diseases, including frontotemporal dementia and neuronal ceroid …

Potent New Targets for Autophagy Enhancement to Delay Neuronal Ageing

J Szinyákovics, F Keresztes, EA Kiss, G Falcsik… - Cells, 2023 - mdpi.com
Autophagy is a lysosomal-dependent degradation process of eukaryotic cells responsible
for breaking down unnecessary and damaged intracellular components. Autophagic activity …

PGRN deficiency exacerbates, whereas a brain penetrant PGRN derivative protects, GBA1 mutation-associated pathologies and diseases

X Zhao, Y Lin, B Liou, W Fu, J Jian… - Proceedings of the …, 2023 - National Acad Sciences
Mutations in GBA1, encoding glucocerebrosidase (GCase), cause Gaucher disease (GD)
and are also genetic risks in developing Parkinson's disease (PD). Currently, the approved …

Selective neuronal expression of progranulin is sufficient to provide neuroprotective and anti-inflammatory effects after traumatic brain injury

S Wang, MP Weyer, R Hummel… - Journal of …, 2024 - Springer
Progranulin (PGRN), which is produced in neurons and microglia, is a neurotrophic and anti-
inflammatory glycoprotein. Human loss-of-function mutations cause frontotemporal …

Intrinsic link between PGRN and Gba1 D409V mutation dosage in potentiating Gaucher disease

Y Lin, X Zhao, B Liou, V Fannin, W Zhang… - Human Molecular …, 2024 - academic.oup.com
Gaucher disease (GD) is caused by biallelic GBA1/Gba1 mutations that encode defective
glucocerebrosidase (GCase). Progranulin (PGRN, encoded by GRN/Grn) is a modifier of …

Progranulin Protects against Aspergillus fumigatus Keratitis by Attenuating the Inflammatory Response through Enhancing Autophagy

P Qi, X Liu, C Li, Q Xu, L Hu, H Duan… - ACS Infectious …, 2024 - ACS Publications
Fungal keratitis (FK) is a severe corneal condition caused by pathogenic fungi and is
associated with the virulence of fungi and an excessive tissue inflammatory response …

Interaction with ERp57 is required for progranulin protection against Type 2 Gaucher disease

Y Liu, X Zhao, J Jian, S Hasan, C Liu - Bioscience trends, 2023 - jstage.jst.go.jp
Gaucher disease (GD), one of the most common lysosomal storage diseases, is caused by
GBA1 mutations resulting in defective glucocerebrosidase (GCase) and consequent …

[HTML][HTML] Natural history of inflammation and impaired autophagy in children with Gaucher disease identified by newborn screening

V Gragnaniello, D Gueraldi, A Saracini… - Molecular Genetics and …, 2025 - Elsevier
Introduction Gaucher disease is a lysosomal storage disease due to deficiency of
glucocerebrosidase, leading to the accumulation of glucosylceramide, particularly in …