Oxidative phosphorylation, ie, ATP synthesis by the oxygen-consuming respiratory chain
(RC), supplies most organs and tissues with a readily usable energy source, being
functional before birth. Consequently, RC deficiencies can theoretically give rise to any
symptom, in any organ or tissue, at any age and with any mode of inheritance, because of
the twofold genetic origin of RC components (nuclear DNA and mitochondrial DNA). It was
long wrongly considered that RC disorders originate from mutations of mitochondrial DNA …