Interplay of RNA-binding proteins and microRNAs in neurodegenerative diseases

C Kinoshita, N Kubota, K Aoyama - International Journal of Molecular …, 2021 - mdpi.com
The number of patients with neurodegenerative diseases (NDs) is increasing, along with the
growing number of older adults. This escalation threatens to create a medical and social …

Pathomechanism heterogeneity in the amyotrophic lateral sclerosis and frontotemporal dementia disease spectrum: providing focus through the lens of autophagy

RL Casterton, RJ Hunt, M Fanto - Journal of molecular biology, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) constitute
aggressive neurodegenerative pathologies that lead to the progressive degeneration of …

The interplay of RNA binding proteins, oxidative stress and mitochondrial dysfunction in ALS

J Harley, BE Clarke, R Patani - Antioxidants, 2021 - mdpi.com
RNA binding proteins fulfil a wide number of roles in gene expression. Multiple mechanisms
of RNA binding protein dysregulation have been implicated in the pathomechanisms of …

Obatoclax Rescues FUS-ALS Phenotypes in iPSC-Derived Neurons by Inducing Autophagy

CM Castillo Bautista, K Eismann, M Gentzel, S Pelucchi… - Cells, 2023 - mdpi.com
Aging is associated with the disruption of protein homeostasis and causally contributes to
multiple diseases, including amyotrophic lateral sclerosis (ALS). One strategy for restoring …

Neuro‐Cells therapy improves motor outcomes and suppresses inflammation during experimental syndrome of amyotrophic lateral sclerosis in mice

JPJM de Munter, I Shafarevich… - CNS neuroscience & …, 2020 - Wiley Online Library
Abstract Aims Mutations in DNA/RNA‐binding factor (fused‐in‐sarcoma) FUS and
superoxide dismutase‐1 (SOD‐1) cause amyotrophic lateral sclerosis (ALS). They were …

Aggregates of RNA binding proteins and ER chaperones linked to exosomes in granulovacuolar degeneration of the Alzheimer's disease brain

A Yamoah, P Tripathi, A Sechi, C Köhler… - Journal of …, 2020 - content.iospress.com
Granulovacuolar degeneration (GVD) occurs in Alzheimer's disease (AD) brain due to
compromised autophagy. Endoplasmic reticulum (ER) function and RNA binding protein …

Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in FUS ALS Motoneurons

VL Zimyanin, AM Pielka, H Glaß, J Japtok, D Großmann… - Cells, 2023 - mdpi.com
Motoneurons are one of the most energy-demanding cell types and a primary target in
Amyotrophic lateral sclerosis (ALS), a debilitating and lethal neurodegenerative disorder …

Aberrant phase separation of FUS leads to lysosome sequestering and acidification

F Trnka, C Hoffmann, H Wang, R Sansevrino… - Frontiers in cell and …, 2021 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that leads
to the death of upper and lower motor neurons. While most cases of ALS are sporadic, some …

Exploring motor neuron diseases using iPSC platforms

AE Johns, NJ Maragakis - Stem Cells, 2022 - academic.oup.com
The degeneration of motor neurons is a pathological hallmark of motor neuron diseases
(MNDs), but emerging evidence suggests that neuronal vulnerability extends well beyond …

Pathomechanisms of ALS8: altered autophagy and defective RNA binding protein (RBP) homeostasis due to the VAPB P56S mutation

P Tripathi, H Guo, A Dreser, A Yamoah, A Sechi… - Cell death & …, 2021 - nature.com
Mutations in RNA binding proteins (RBPs) and in genes regulating autophagy are frequent
causes of familial amyotrophic lateral sclerosis (fALS). The P56S mutation in vesicle …