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Updated: Apr 27, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess
Published on: June 1, 2022
Cell-permeable protein therapy for complex I dysfunction
Salvatore Pepe1, Robert M Mentzer, Roberta A Gottlieb
1Heart Research, Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Australia.
Abstract:
Complex I deficiency is difficult to treat because of the size and complexity of the multi-subunit enzyme complex. Mutations or deletions in the mitochondrial genome are not amenable to gene therapy. However, animal studies have shown that yeast-derived internal NADH quinone oxidoreductase (Ndi1) can be delivered as a cell-permeable recombinant protein (Tat-Ndi1) that can functionally replace complex I damaged by ischemia/reperfusion. Current and future treatment of disorders affecting complex I are discussed, including the use of Tat-Ndi1.
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