Crossing UCHL1-eGFP with hSOD1G93A mice, we generated the hSOD1G93A-UeGFP transgenic mouse model of ALS, and showed that this disease reporter recapitulates previously reported CSMN degeneration in the hSOD1G93A mice [18,39]. The gene discussed is UCHL1; the disease is amyotrophic lateral sclerosis.