It would be interesting to determine if: (i) endogenous ADAMTS-4 contributes to neurodegeneration in mice expressing high copy number of mutant SOD1 as well as in other model of ALS such as WT or mutant TDP43 (TAR DNA-binding protein 43) rodent models, or even in frontotemporal dementia or AD models; (ii) therapeutic approaches aimed at decreasing ADAMTS-4 expression/activity would represent potential targets to slow down neurodegeneration in chronic CNS diseases. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.