Lysosomal dysfunction in neurodegeneration: emerging concepts and methods

V Udayar, Y Chen, E Sidransky, R Jagasia - Trends in neurosciences, 2022 - cell.com
The understanding of lysosomes has come a long way since the initial discovery of their role
in degrading cellular waste. The lysosome is now recognized as a highly dynamic organelle …

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 …

Genome-wide CRISPRi/a screens in human neurons link lysosomal failure to ferroptosis

R Tian, A Abarientos, J Hong, SH Hashemi, R Yan… - Nature …, 2021 - nature.com
Single-cell transcriptomics provide a systematic map of gene expression in different human
cell types. The next challenge is to systematically understand cell-type-specific gene …

Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic

T Logan, MJ Simon, A Rana, GM Cherf, A Srivastava… - Cell, 2021 - cell.com
GRN mutations cause frontotemporal dementia (GRN-FTD) due to deficiency in progranulin
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …

RNA granules hitchhike on lysosomes for long-distance transport, using annexin A11 as a molecular tether

YC Liao, MS Fernandopulle, G Wang, H Choi, L Hao… - Cell, 2019 - cell.com
Long-distance RNA transport enables local protein synthesis at metabolically-active sites
distant from the nucleus. This process ensures an appropriate spatial organization of …

[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 …

An overview of the role of lipofuscin in age-related neurodegeneration

A Moreno-García, A Kun, O Calero, M Medina… - Frontiers in …, 2018 - frontiersin.org
Despite aging being by far the greatest risk factor for highly prevalent neurodegenerative
disorders, the molecular underpinnings of age-related brain changes are still not well …

[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 …

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1

DJ Klionsky, AK Abdel-Aziz, S Abdelfatah, M Abdellatif… - autophagy, 2021 - Taylor & Francis
In 2008, we published the first set of guidelines for standardizing research in autophagy.
Since then, this topic has received increasing attention, and many scientists have entered …

[HTML][HTML] Guidelines for the use and interpretation of assays for monitoring autophagy 1

DJ Klionsky, AK Abdel-Aziz, S Abdelfatah… - …, 2021 - pmc.ncbi.nlm.nih.gov
In 2008, we published the first set of guidelines for standardizing research in autophagy.
Since then, this topic has received increasing attention, and many scientists have entered …