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Given the complexity of mitochondrial genetics and biochemistry, mitochondrial inherited diseases may present with a vast range of symptoms, organ involvement, severity, age of onset, and outcome. Despite the wide spectrum of clinical signs and biochemical underpinnings of this group of dis\u2010orders, some common traits can be identified, based on both pathogenic mechanisms and potential therapeutic approaches. Here, we will review two peculiar mitochondrial disorders, ethylmalonic encephalopathy (EE) and mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), caused by mutations in the\n                    <jats:italic>ETHE1<\/jats:italic>\n                    and\n                    <jats:italic>TYMP<\/jats:italic>\n                    nuclear genes, respectively.\n                    <jats:italic>ETHE1<\/jats:italic>\n                    encodes for a mitochondrial enzyme involved in sulfide detoxification and\n                    <jats:italic>TYMP<\/jats:italic>\n                    for a cytosolic enzyme involved in the thymidine\/deoxyuridine catabolic pathway. We will discuss these two clinical entities as a paradigm of mitochondrial diseases caused by the accumulation of compounds normally present in traces, which exerts a toxic and inhibitory effect on the OXPHOS system.\n                  <\/jats:p>","DOI":"10.15252\/emmm.201505040","type":"journal-article","created":{"date-parts":[[2015,7,20]],"date-time":"2015-07-20T21:15:19Z","timestamp":1437426919000},"page":"1257-1266","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Mitochondrial diseases caused by toxic compound accumulation: from etiopathology to therapeutic approaches"],"prefix":"10.1038","volume":"7","author":[{"given":"Ivano","family":"Di Meo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Costanza","family":"Lamperti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Tiranti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,7,20]]},"reference":[{"key":"BFEMMM201505040_CR1","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1523\/JNEUROSCI.16-03-01066.1996","volume":"16","author":"K Abe","year":"1996","unstructured":"Abe K, Kimura H (1996) The possible role of hydrogen sulfide as an endogenous neuromodulator. 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