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However, recent technological advances including somatic cell reprogramming to induced pluripotent stem cells (iPSCs), organoid technology, and tailored endonucleases provide unprecedented opportunities to fill these gaps, casting hope to soon cure the major primary mitochondrial phenotypes reviewed here. This group of rare diseases represents a key model for tackling the pathogenic mechanisms involving mitochondrial biology relevant to much more common disorders that affect our currently ageing population, such as diabetes and metabolic syndrome, neurodegenerative and inflammatory disorders, and cancer.<\/jats:p>","DOI":"10.1042\/ebc20170097","type":"journal-article","created":{"date-parts":[[2018,7,20]],"date-time":"2018-07-20T10:35:16Z","timestamp":1532082916000},"page":"235-254","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Clinical syndromes associated with mtDNA mutations: where we stand after 30 years"],"prefix":"10.1042","volume":"62","author":[{"given":"Valerio","family":"Carelli","sequence":"additional","affiliation":[{"name":"IRCCS Institute of Neurological Sciences of Bologna, Bellaria Hospital, Bologna, Italy"},{"name":"Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"La Morgia","sequence":"additional","affiliation":[{"name":"IRCCS Institute of Neurological Sciences of Bologna, Bellaria Hospital, Bologna, Italy"},{"name":"Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2018,7,20]]},"reference":[{"key":"2021111610255893600_B1","doi-asserted-by":"publisher","first-page":"1776","DOI":"10.1172\/JCI104637","article-title":"A case of severe hypermetabolism of nonthyroid origin with a defect in the maintenance of mitochondrial respiratory control: a correlated clinical, biochemical, and morphological study","volume":"41","author":"Luft","year":"1962","journal-title":"J. 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