Ferraiuolo et al. (2011) have shown that the increase in total NGF is due to the secreted pro-NGF fraction before cleavage to the mature form, achieving a twofold increased ratio in the SOD1G93 mice as compared to control conditions. Such finding may derive from the increased ALS-associated MMP-9 (Niebroj-Dobosz et al., 2010), one of the enzymes that degrade mature NGF in the extracellular space. Here, NGF is linked to amyotrophic lateral sclerosis.