Amyotrophic Lateral Sclerosis Involving Gliopathy

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease, causing death within two to five years following diagnosis. ALS is a disease characterized by motor neuron degeneration and destruction of the neuromuscular junction (NMJ). To date, numerous genetic mutations have been associated with ALS, but the mutation in the gene coding for superoxide dismutase (SOD1) has been the most extensively studied. Nevertheless, studies carried out on rodent models and ALS patients have shown that damage to glial cells contributes directly to the development and progression of the disease. However, few studies have focused on the properties of glial cells in ALS. The accumulation of knowledge on the active role and pathological mechanisms of each glial type in the disease must be carefully applied to better understand the ALS pathophysiology, but also for the development of targeted therapy for glial cells. Therefore, in this chapter the authors aimed to compile the most recent information on how each type of glial cell contributes to the development of ALS.