Topic: motor neuron death

The clinical manifestations of sporadic amyotrophic lateral sclerosis (ALS) are highly variable, implying existence of subgroups in pathophysiology. We studied a group of patients with sporadic ALS and an ALS mouse model having superoxide dismutase 1 (SOD1)/G93A transgenes using chemical precipitation followed by enzyme-linked immunosorbent assay analysis for measurement of plasma misfolded protein levels and by a nerve excitability test (NET) to investigate axonal membrane properties. Six of 19 patients (31.6%) with sporadic ALS had elevated plasma misfolded SOD1 protein levels. In sporadic A

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