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Much remains to be done, however, and this brief review discusses the following areas of research where progress has been more limited or data are still controversial: (1) the molecular basis of respiratory\u2010chain defects due to nuclear DNA mutations; (2) defects of mitochondrial protein importation; (3) defects of intergenomic signalling; (4) pathophysiology of mtDNA\u2010related disorders; (5) ageing and age\u2010related neuro\u2010degenerative diseases; (6) therapy; and (7) genetic counselling.<\/jats:p>","DOI":"10.1007\/bf01799110","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T21:01:28Z","timestamp":1119906088000},"page":"489-503","source":"Crossref","is-referenced-by-count":44,"title":["Mitochondrial encephalomyopathies: what next?"],"prefix":"10.1002","volume":"19","author":[{"given":"S.","family":"DiMauro","sequence":"first","affiliation":[{"name":"H. 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