Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations

► SURF1 mutations are frequent cause of severe COX deficiency. ► Altered energy provision leads to increase of respiratory chain complexes I, III, and V. ► Adaptive changes in mitochondrial biogenesis are due to posttranscriptional events. ► All residual COX is incorporated into I-III2-IV1 supercomplex.