Another major human disorder that is linked to the Mia40 pathway is ALS (Amyotrophic lateral sclerosis), which is known to be associated with mutations of SOD1 [130], [131], [132], that cannot form the disulfide bonds properly and become aggregated leading to impairment of the mitochondrial function. The gene discussed is CHCHD4; the disease is amyotrophic lateral sclerosis.