Second, substrates of the MIA pathway are linked also to primary mitochondrial diseases (such as several mitochondriopathies related to COX deficiencies) and to more common neurodegenerative diseases (such as for example CHCHD2 and CHCHD10, which are linked to amyotrophic lateral sclerosis) [100]. Here, CHCHD2 is linked to inborn mitochondrial metabolism disorder.