Nuclear DNA encodes over 1000 mitochondrial-localized proteins [15], and while the majority of variants in nuclear-encoded mitochondrial genes are inherited in a recessive manner, there are some cases of dominantly inherited mitochondrial disease, such as paragangliomas associated with mutations in SDHC (succinate dehydrogenase complex subunit C) [13,16]. Here, SDHC is linked to mitochondrial disease.