SOD1G93A

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurological disorder characterized by the gradual degeneration of cells in the spinal cord, brainstem, and motor cortex, leading to manifestations such as weakness, muscle atrophy, and spasticity (Elisen, 2009). A comprehensive understanding of the disease process is currently lacking, making preclinical ALS models crucial to study the mechanisms of pathology insurgence, characterize the disease and test therapeutics approaches. Pathological in vitro models emerged as viable alternative to in vivo studies, consolidating a non-invasive appro

…objective of this study in the murine SOD1G93A A

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease that causes the degeneration of motor neurons in the motor cortex and spinal cord. Patients with ALS experience muscle weakness and atrophy in the limbs which eventually leads to paralysis and death. NAD+ is critical for energy metabolism, such as glycolysis and oxidative phosphorylation, but is also involved in non-metabolic cellular reactions. In the current study, we determined whether the supplementation of nicotinamide mononucleotide (NMN), an NAD+ precursor, in the diet had beneficial impacts on disease progr

…oxidative/nitrosative stress in SOD1G93A ALS mice

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by motor neuron death, leading to motor paralysis and cognitive and behavioural disorders. In clinical trials, ALS patients are stratified by disease stage, whereas in preclinical studies, animals are recruited based solely on age, neglecting individual disease progression. Additionally, both ALS onset and progression are influenced by sex, a factor often mirrored in animal models. This mismatch might contribute to the frequent failures of translating preclinical data to clinical success, suggesting the impo