SOD1 and amyotrophic lateral sclerosis: Progesterone might delay the neurodegeneration process by activating autophagy degradation of mutant SOD1. On the other hand, Heitzer et al. [81] revealed that in male SOD1 G93A ALS mice, the treatment with 17β-Estradiol increases the survival of motoneurons, suggesting that this could be related to the downregulation of several components of the inflammatory response (e.g., NLRP3, IL1beta, and activated caspase 1) abnormally elevated in the spinal cord of the affected mice [81].