Mutations in SDHAF1 and SDHA lead to encephalomyopathy and leukoencephalopathy [184,188], while variants in SDHA, SDHB, SDHC, SDHD and SDHAF2 are responsible for hereditary paraganglioma and pheochromocytomas, rare neuroendocrine tumors [183,189,190,191,192]. Here, SDHA is linked to mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria.