Interestingly, MCU is downregulated in motor neurons of asymptomatic or early-disease stage mice expressing ALS-linked SOD1 but is upregulated in the surviving neurons of late-stage animals, suggesting that those neurons that maintain the highest mitochondrial Ca2+ buffering capacity are the most resilient to cell death253,254. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.