[HTML][HTML] SOD1 in ALS: taking stock in pathogenic mechanisms and the role of glial and muscle cells

C Peggion, V Scalcon, ML Massimino, K Nies… - Antioxidants, 2022 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
the loss of motor neurons in the brain and spinal cord. While the exact causes of ALS are still …

[HTML][HTML] Swimming against ALS: how to model disease in zebrafish for pathophysiological and behavioral studies

NAS Oliveira, BR Pinho, JMA Oliveira - Neuroscience & Biobehavioral …, 2023 - Elsevier
Abstract Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that leads to
progressive disability and motor impairment. Existing therapies provide modest …

[HTML][HTML] Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling

C Lasbleiz, A Peyrel, P Tarot, J Sarniguet, L Crouzier… - Redox Biology, 2022 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting upper and
lower motor neurons. As a consequence, ALS patients display a locomotor disorder related …

[HTML][HTML] Neuroimmune characterization of optineurin insufficiency mouse model during ageing

N Mohovic, J Peradinovic, A Markovinovic, R Cimbro… - Scientific Reports, 2023 - nature.com
Optineurin is a multifunctional polyubiquitin-binding protein implicated in inflammatory
signalling. Optineurin mutations are associated with amyotrophic lateral sclerosis (ALS) and …

[HTML][HTML] Reactive gliosis in traumatic brain injury: a comprehensive review

Z Amlerova, M Chmelova, M Anderova… - Frontiers in Cellular …, 2024 - frontiersin.org
Traumatic brain injury (TBI) is one of the most common pathological conditions impacting the
central nervous system (CNS). A neurological deficit associated with TBI results from a …

Zebrafish as a model organism for studying pathologic mechanisms of neurodegenerative diseases and other neural disorders

Y Liu - Cellular and Molecular Neurobiology, 2023 - Springer
Zebrafish are widely considered an excellent vertebrate model for studying the
pathogenesis of human diseases because of their transparency of embryonic development …

[HTML][HTML] The role and therapeutic potential of the integrated stress response in amyotrophic lateral sclerosis

E Marlin, C Viu-Idocin, M Arrasate… - International journal of …, 2022 - mdpi.com
In amyotrophic lateral sclerosis (ALS) patients, loss of cellular homeostasis within cortical
and spinal cord motor neurons triggers the activation of the integrated stress response (ISR) …

Pharmacological inhibition of the integrated stress response accelerates disease progression in an amyotrophic lateral sclerosis mouse model

E Marlin, M Valencia, N Peregrín… - British Journal of …, 2024 - Wiley Online Library
Background and Purpose The integrated stress response (ISR) regulates translation in
response to diverse stresses. ISR activation has been documented in amyotrophic lateral …

[HTML][HTML] Therapeutic targets in amyotrophic lateral sclerosis: focus on ion channels and skeletal muscle

N Tarantino, I Canfora, GM Camerino, S Pierno - Cells, 2022 - mdpi.com
Amyotrophic Lateral Sclerosis is a neurodegenerative disease caused by progressive loss
of motor neurons, which severely compromises skeletal muscle function. Evidence shows …

[HTML][HTML] Edaravone counteracts redox and metabolic disruptions in an emerging zebrafish model of sporadic ALS

NAS Oliveira, BR Pinho, J Pinto, PG de Pinho… - Free Radical Biology …, 2024 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which the death of
motor neurons leads to loss of muscle function. Additionally, cognitive and circadian …