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 …

[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis

J Root, P Merino, A Nuckols, M Johnson… - Neurobiology of disease, 2021 - Elsevier
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …

Aggregation-prone c9FTD/ALS poly (GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

YJ Zhang, K Jansen-West, YF Xu, TF Gendron… - Acta …, 2014 - Springer
The occurrence of repeat-associated non-ATG (RAN) translation, an atypical form of
translation of expanded repeats that results in the synthesis of homopolymeric expansion …

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 …

The lysosomal function of progranulin, a guardian against neurodegeneration

DH Paushter, H Du, T Feng, F Hu - Acta neuropathologica, 2018 - Springer
Progranulin (PGRN), encoded by the GRN gene in humans, is a secreted growth factor
implicated in a multitude of processes ranging from regulation of inflammation to wound …

Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis

ME Ward, R Chen, HY Huang, C Ludwig… - Science translational …, 2017 - science.org
Heterozygous mutations in the GRN gene lead to progranulin (PGRN) haploinsufficiency
and cause frontotemporal dementia (FTD), a neurodegenerative syndrome of older adults …

Autophagy induction by trehalose: Molecular mechanisms and therapeutic impacts

K Hosseinpour‐Moghaddam… - Journal of cellular …, 2018 - Wiley Online Library
The balance between synthesis and degradation is crucial to maintain cellular homeostasis
and different mechanisms are known to keep this balance. In this review, we will provide a …

Insights into the role of progranulin in immunity, infection, and inflammation

J Jian, J Konopka, C Liu - Journal of leukocyte biology, 2013 - academic.oup.com
Review on the role of progranulin in immunity, infection, and inflammation, and its
therapeutic potential in treating inflammatory conditions. PGRN, a pleiotrophic growth factor …

Disaccharide trehalose in experimental therapies for neurodegenerative disorders: Molecular targets and translational potential

AB Pupyshev, TP Klyushnik, AA Akopyan… - Pharmacological …, 2022 - Elsevier
Induction of autophagy is a prospective approach to the treatment of neurodegeneration. In
the recent decade, trehalose attracted special attention. It is an autophagy inducer with …

Progranulin gene therapy improves lysosomal dysfunction and microglial pathology associated with frontotemporal dementia and neuronal ceroid lipofuscinosis

AE Arrant, VC Onyilo, DE Unger… - Journal of …, 2018 - Soc Neuroscience
Loss-of-function mutations in progranulin, a lysosomal glycoprotein, cause
neurodegenerative disease. Progranulin haploinsufficiency causes frontotemporal dementia …