Neuromuscular junction dysfunction in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
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 …
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 …
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 …
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 …
human neuropathology. The zebrafish brain exhibits fundamental resemblance with human …
Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic
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 …
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 …
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 …
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)
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 …
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 …
dementia (FTD) is a hexanucleotide repeat expansion within the C9orf72 gene. Reduced …