Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …

Molecular and cellular mechanisms affected in ALS

L Le Gall, E Anakor, O Connolly… - Journal of personalized …, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …

Modelling amyotrophic lateral sclerosis: progress and possibilities

P Van Damme, W Robberecht… - Disease models & …, 2017 - journals.biologists.com
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that primarily affects the
motor system and presents with progressive muscle weakness. Most patients survive for …

Advances of zebrafish in neurodegenerative disease: from models to drug discovery

X Wang, JB Zhang, KJ He, F Wang… - Frontiers in Pharmacology, 2021 - frontiersin.org
Neurodegenerative disease (NDD), including Alzheimer's disease, Parkinson's disease, and
amyotrophic lateral sclerosis, are characterized by the progressive loss of neurons which …

Zebrafish as a model organism for neurodegenerative disease

K Chia, A Klingseisen, D Sieger… - Frontiers in molecular …, 2022 - frontiersin.org
The zebrafish is increasingly recognized as a model organism for translational research into
human neuropathology. The zebrafish brain exhibits fundamental resemblance with human …

Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic

BG Trist, JB Hilton, DJ Hare, PJ Crouch… - Angewandte Chemie …, 2021 - Wiley Online Library
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …

From animal models to human disease: a genetic approach for personalized medicine in ALS

V Picher-Martel, PN Valdmanis, PV Gould… - Acta neuropathologica …, 2016 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is the most frequent motor neuron disease in
adults. Classical ALS is characterized by the death of upper and lower motor neurons …

[HTML][HTML] Zebrafish models of human motor neuron diseases: advantages and limitations

PJ Babin, C Goizet, D Raldúa - Progress in neurobiology, 2014 - Elsevier
Motor neuron diseases (MNDs) are an etiologically heterogeneous group of disorders of
neurodegenerative origin, which result in degeneration of lower (LMNs) and/or upper motor …

Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS)

S Sangwan, A Zhao, KL Adams… - Proceedings of the …, 2017 - National Acad Sciences
Fibrils and oligomers are the aggregated protein agents of neuronal dysfunction in ALS
diseases. Whereas we now know much about fibril architecture, atomic structures of disease …

Reduced C9orf72 function leads to defective synaptic vesicle release and neuromuscular dysfunction in zebrafish

Z Butti, YE Pan, J Giacomotto, SA Patten - Communications biology, 2021 - nature.com
The most common genetic cause of amyotrophic lateral sclerosis (ALS) and fronto-temporal
dementia (FTD) is a hexanucleotide repeat expansion within the C9orf72 gene. Reduced …