When hSOD1G93A mice—the golden standard for ALS drug discovery efforts for the past 15 years—are crossed with UCHL1-eGFP reporter mice to generate hSOD1G93A-UeGFP ALS reporter mouse model, a significant reduction in the number CSMN was observed, but in the spinal cord, eGFP expression was restricted mostly to S and gamma SMN resistant to degeneration in ALS for unknown reasons [20]. This evidence concerns the gene UCHL1 and amyotrophic lateral sclerosis.