[HTML][HTML] Lysosomal functions of progranulin and implications for treatment of frontotemporal dementia

MJ Simon, T Logan, SL DeVos, G Di Paolo - Trends in Cell Biology, 2023 - cell.com
Loss-of-function heterozygous mutations in GRN, the gene encoding progranulin (PGRN),
were identified in patients with frontotemporal lobar degeneration (FTLD) almost two …

Prosaposin maintains lipid homeostasis in dopamine neurons and counteracts experimental parkinsonism in rodents

Y He, I Kaya, R Shariatgorji, J Lundkvist… - Nature …, 2023 - nature.com
Prosaposin (PSAP) modulates glycosphingolipid metabolism and variants have been linked
to Parkinson's disease (PD). Here, we find altered PSAP levels in the plasma, CSF and post …

Regulation of lysosomal trafficking of progranulin by sortilin and prosaposin

H Du, X Zhou, T Feng, F Hu - Brain Communications, 2022 - academic.oup.com
Haploinsufficiency of the progranulin protein is a leading cause of frontotemporal lobar
degeneration. Accumulating evidence support a crucial role of progranulin in the lysosome …

Progranulin deficiency results in sex-dependent alterations in microglia in response to demyelination

T Zhang, T Feng, K Wu, J Guo, AL Nana, G Yang… - Acta …, 2023 - Springer
Heterozygous mutations in the granulin (GRN) gene, resulting in the haploinsufficiency of
the progranulin (PGRN) protein, is a leading cause of frontotemporal lobar degeneration …

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 …

Benzoxazole-derivatives enhance progranulin expression and reverse the aberrant lysosomal proteome caused by GRN haploinsufficiency

R Tesla, C Guhl, GC Werthmann, D Dixon… - Nature …, 2024 - nature.com
Heterozygous loss-of-function mutations in the GRN gene are a major cause of hereditary
frontotemporal dementia. The mechanisms linking frontotemporal dementia pathogenesis to …

Charge and redox states modulate granulin—TDP-43 coacervation toward phase separation or aggregation

AA Bhopatkar, S Dhakal, HG Abernathy, SE Morgan… - Biophysical journal, 2022 - cell.com
Cytoplasmic inclusions containing aberrant proteolytic fragments of TDP-43 are associated
with frontotemporal lobar degeneration (FTLD) and other related pathologies. In FTLD, TDP …

Progranulin from different gliocytes in the nucleus accumbens exerts distinct roles in FTD-and neuroinflammation-induced depression-like behaviors

J Wang, S Lai, T Zhou, Z Xia, W Li, W Sha, J Liu… - Journal of …, 2022 - Springer
Background Neuroinflammation in the nucleus accumbens (NAc) is well known to influence
the progression of depression. However, the molecular mechanisms triggering NAc …

Patients with sporadic FTLD exhibit similar increases in lysosomal proteins and storage material as patients with FTD due to GRN mutations

SE Davis, AK Cook, JA Hall, Y Voskobiynyk… - Acta neuropathologica …, 2023 - Springer
Loss of function progranulin (GRN) mutations are a major autosomal dominant cause of
frontotemporal dementia (FTD). Patients with FTD due to GRN mutations (FTD-GRN) …

[HTML][HTML] Granulins rescue inflammation, lysosome dysfunction, and neuropathology in a mouse model of progranulin deficiency

J Root, A Mendsaikhan, S Nandy, G Taylor, M Wang… - bioRxiv, 2023 - ncbi.nlm.nih.gov
Progranulin (PGRN) deficiency is linked to neurodegenerative diseases including
frontotemporal dementia, Alzheimer's disease, Parkinson's disease, and neuronal ceroid …