Transcriptional changes in OXPHOS complex I deficiency are related to anti-oxidant pathways and could explain the disturbed calcium homeostasis

► Defective complex I (CI) is the most common type of oxidative phosphorylation disease. ► Transcriptomics identified novel molecular processes underlying CI deficiency in fibroblasts of patients. ► The Nrf2 pathway is induced in fibroblasts of complex I deficient patients. ► Disturbed calcium homeostasis in complex I deficiency can be related to selenoproteins. ► Glutathione and selenium metabolism are potentially therapeutic targets in CI deficiency.