Given that a significant portion of the MMP-9 is in its active state and capable of cleaving proteins such as myelin basic protein, matrix proteins and activating various inflammatory cytokines, it is most probable that it is a significant source of further inflammatory activation, providing a compelling rationale for its inhibition not only in the CNS, but systemically, with an inhibitor such as AQU-118 as a potential approach to reducing ALS induced muscle degeneration. Here, MMP9 is linked to amyotrophic lateral sclerosis.