Amyotrophic lateral sclerosis (ALS) is a prototypical neurodegenerative disease that is characterized by the progressive degeneration of motor neurons in the brain and spinal cord. Its clinical hallmark is the degeneration of both upper and lower motor neurons, leading to progressive muscle atrophy, weakness, and paralysis.
Although the cause is still unknown, several genetic and molecular pathways are involved in the development and progression of ALS. These mechanisms include altered RNA processing leading to prion-like self-aggregation, superoxide dismutase type 1 SOD1 mutations leading to free radical toxicity, cascading inflammatory responses, excessive concentrations of glutamate, mitochondrial dysfunction, and disruption of axonal transport processes. The recently identified hexanucleotide repeat expansion in the noncoding region of the chromosome 9 open reading frame 72 gene (c9orf72RE) is another common genetic cause of ALS spectrum disorder (Todd, 2016).
We here at 2B Scientific supply tools to discover potential ALS causes, clinical trials, and drug discovery.