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Primary mitochondrial diseases are mainly caused by defects in one or several components of the oxidative phosphorylation system (complexes I\u2013V). Within these disorders, those associated with complex III deficiencies are the least common. However, thanks to a deeper knowledge about complex III biogenesis, improved clinical diagnosis and the implementation of next\u2010generation sequencing techniques, the number of pathological variants identified in nuclear genes causing complex III deficiency has expanded significantly. 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