Endo-lysosomal dysfunction in neurodegenerative diseases: Opinion on current progress and future direction in the use of exosomes as biomarkers

M Herman, GW Randall, JL Spiegel… - … of the Royal …, 2024 - royalsocietypublishing.org
Over the past two decades, increased research has highlighted the connection between
endosomal trafficking defects and neurodegeneration. The endo-lysosomal network is an …

[HTML][HTML] Dysregulation of the progranulin-driven autophagy-lysosomal pathway mediates secretion of the nuclear protein TDP-43

Y Tanaka, S Ito, Y Honma, M Hasegawa… - Journal of Biological …, 2023 - ASBMB
The cytoplasmic accumulation of the nuclear protein transactive response DNA-binding
protein 43 kDa (TDP-43) has been linked to the progression of amyotrophic lateral sclerosis …

CKLF induces microglial activation via triggering defective mitophagy and mitochondrial dysfunction

H Wang, J Ye, Y Peng, W Ma, H Chen, H Sun, Z Feng… - Autophagy, 2024 - Taylor & Francis
Although microglial activation is induced by an increase in chemokines, the role of
mitophagy in this process remains unclear. This study aimed to elucidate the role of …

[HTML][HTML] New insights and therapeutic opportunities for progranulin-deficient frontotemporal dementia

S Amin, G Carling, L Gan - Current Opinion in Neurobiology, 2022 - Elsevier
Frontotemporal dementia (FTD) is the second most common form of dementia. It affects the
frontal and temporal lobes of the brain and has a highly heterogeneous clinical …

[HTML][HTML] 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 …

[HTML][HTML] Emerging roles for phase separation of RNA-binding proteins in cellular pathology of ALS

K Milicevic, B Rankovic, PR Andjus… - Frontiers in Cell and …, 2022 - frontiersin.org
Liquid-liquid phase separation (LLPS) is emerging as a major principle for the mesoscale
organization of proteins, RNAs, and membrane-bound organelles into biomolecular …

Can we treat neurodegenerative proteinopathies by enhancing protein degradation?

S Engelender, L Stefanis, S Oddo… - Movement …, 2022 - Wiley Online Library
Neurodegenerative proteinopathies are defined as a class of neurodegenerative disorders,
with either genetic or sporadic age‐related onset, characterized by the pathological …

[HTML][HTML] Drosophila melanogaster as a model to study autophagy in neurodegenerative diseases induced by proteinopathies

S Santarelli, C Londero, A Soldano… - Frontiers in …, 2023 - frontiersin.org
Proteinopathies are a large group of neurodegenerative diseases caused by both genetic
and sporadic mutations in particular genes which can lead to alterations of the protein …

Neurotropic virus infection and neurodegenerative diseases: Potential roles of autophagy pathway

Y Zhao, K Xu, F Shu, F Zhang - CNS Neuroscience & …, 2024 - Wiley Online Library
Neurodegenerative diseases (NDs) constitute a group of disorders characterized by the
progressive deterioration of nervous system functionality. Currently, the precise etiological …

[HTML][HTML] A novel CRISPR/Cas9-based iduronate-2-sulfatase (IDS) knockout human neuronal cell line reveals earliest pathological changes

L Badenetti, R Manzoli, M Trevisan, F D'Avanzo… - Scientific Reports, 2023 - nature.com
Multiple complex intracellular cascades contributing to Hunter syndrome (
mucopolysaccharidosis type II) pathogenesis have been recognized and documented in the …