Copper toxicity and deficiency: the vicious cycle at the core of protein aggregation in ALS

JH Min, RA Harris, H Sarlus - Frontiers in Molecular Neuroscience, 2024 - frontiersin.org
The pathophysiology of ALS involves many signs of a disruption in copper homeostasis, with
both excess free levels and functional deficiency likely occurring simultaneously. This is …

[HTML][HTML] Transcriptomic evaluation of tau and TDP-43 synergism shows tauopathy predominance and reveals potential modulating targets

VS Jadhav, JG Stair, RJ Eck, SN Smukowski… - Neurobiology of …, 2024 - Elsevier
Abstract Alzheimer's disease (AD), the most common aging-associated neurodegenerative
dementia disorder, is defined by the presence of amyloid beta (Aβ) and tau aggregates in …

Defects of nutrient signaling and autophagy in neurodegeneration

J Ondaro, H Hernandez-Eguiazu… - Frontiers in Cell and …, 2022 - frontiersin.org
Neurons are post-mitotic cells that allocate huge amounts of energy to the synthesis of new
organelles and molecules, neurotransmission and to the maintenance of redox …

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 …

Molecular pathways involved in frontotemporal lobar degeneration with TDP-43 proteinopathy: what can we learn from proteomics?

MO Mol, SSM Miedema, JC van Swieten… - International Journal of …, 2021 - mdpi.com
Frontotemporal lobar degeneration (FTLD) is a neurodegenerative disorder clinically
characterized by behavioral, language, and motor symptoms, with major impact on the lives …

Immune signaling kinases in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

R García-García, L Martín-Herrero… - International Journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disorder of
motor neurons in adults, with a median survival of 3–5 years after appearance of symptoms …

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 …

Enhancement of lysosome biogenesis as a potential therapeutic approach for neurodegenerative diseases

W Xue, J Zhang, Y Li - Neural Regeneration Research, 2023 - journals.lww.com
Millions of people are suffering from Alzheimer's disease globally, but there is still no
effective treatment for this neurodegenerative disease. Thus, novel therapeutic approaches …

The role of lysosomal membrane proteins in autophagy and related diseases

J Xu, J Gu, W Pei, Y Zhang, L Wang, J Gao - The FEBS journal, 2023 - Wiley Online Library
As a self‐degrading and highly conserved survival mechanism, autophagy plays an
important role in maintaining cell survival and recycling. The discovery of autophagy‐related …

Defective axonal transport of endo‐lysosomes and dense core vesicles in a Drosophila model of C9‐ALS/FTD

H Sung, TE Lloyd - Traffic, 2022 - Wiley Online Library
Abstract A GGGGCC (G4C2) repeat expansion in the C9orf72 gene is the most common
genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …