1. Alam NM, Mills WC IV, Wong AA, et al. A mitochondrial therapeutic reverses visual decline in mouse models of diabetes. Dis Model Mech. 2015;8(7):701-710.
  2. Ames A III, Li YY, Heher EC, Kimble CR. Energy metabolism of rabbit retina as related to function: high cost of Na+ transport. J Neurosci. 1992;12(3):840-853.
  3. Attwell D, Laughlin SB. An energy budget for signaling in the grey matter of the brain. J Cereb Blood Flow Metab. 2001;21(10):1133-1145.
  4. Bayeva M, Gheorghiade M, Ardehali H. Mitochondria as a therapeutic target in heart failure. J Am Coll Cardiol. 2013;61(6):599-610.
  5. Befroy DE, Rothman DL, Petersen KF, Shulman GI. P-Magnetization transfer magnetic resonance spectroscopy measurements of in vivo metabolism. Diabetes. 2012;61:2669-2678.
  6. Birk AV, Liu S, Soong Y, et al. The mitochondrial-targeted compound SS-31 re-energizes ischemic mitochondria by interacting with cardiolipin. J Am Soc Nephrol. 2013;24(8):1250-1261.
  7. Blei ML, Conley KE, Kushmerick MJ. Separate measures of ATP utilization and recovery in human skeletal muscle. J Physiol. 1993;465:203-222.
  8. Brown DA, Hale SL, Baines CP, et al. Reduction of early reperfusion injury with the mitochondria-targeting peptide Bendavia. J Cardiovasc Pharmacol Ther. 2014;19(1):121-132.
  9. Brown DA, Sabbah HN, Shaikh SR. Mitochondrial inner membrane lipids and proteins as targets for decreasing cardiac ischemia/reperfusion injury. Pharmacol Ther. 2013;140(3):258-266.
  10. Cai M, Li J, Lin S, et al. Mitochondria-targeted antioxidant peptide SS31 protects cultured human lens epithelial cells against oxidative stress. Curr Eye Res. 2015;40(8):822-829.
  11. Carelli V, La Morgia C, Sadun AA. Mitochondrial dysfunction in optic neuropathies: animal models and therapeutic options. Curr Opin Neurol. 2013;26(1):52-58.
  12. Carreras MC, Franco MC, Peralta JG, Poderoso JJ. Nitric oxide, complex I, and the modulation of mitochondrial reactive species in biology and disease. Molecular Aspects Med. 2004:25 (1-2): 125-139.
  13. Chance B, Sies H, Boveris A. Hydroperoxide metabolism in mammalian organs. Physiol Rev. 1979;59(3):527-605.
  14. Chen M, Liu B, Gao Q, Zhuo Y, Ge J. Mitochondria-targeted peptide MTP-131 alleviates mitochondrial dysfunction and oxidative damage in human trabecular meshwork cells. Invest Ophthalmol Vis Sci. 2011;52(10):7027-7037.
  15. Chistiakov DA, Sobenin IA, Revin VV, Orekhov AN, Bobryshev YV. Mitochondrial aging and age-related dysfunction of mitochondria. Biomed Res Int. 2014;2014:238463.
  16. Cousins SC. The rationale for mitochondrial targeted therapeutics in dry AMD. Paper presented at: Angiogenesis, Exudation, and Degeneration 2015; February 7, 2015; Miami, FL.
  17. Cousins SW. Role of mitochondrial dysfunction in dry age-related macular degeneration. Retina Today. June 11, 2015:83-85.
  18. Dai DF, Rabinovitch PS, Ungvari Z. Mitochondria and cardiovascular aging. Circ Res. 2012;110(8):1109-1124.
  19. Daubert MA, Yow E, Dunn G, et al. Effects of a novel tetrapeptide in heart failure with reduced ejection fraction: a phase I randomized, placebo-controlled trial of elamipretide. ACC 64th Annual Scientific Session and Expo; March 16, 2016; Poster 101.
  20. Eirin A, Ebrahimi B, Zhang X, et al. Mitochondrial protection restores renal function in swine atherosclerotic renovascular disease. Cardiovasc Res. 2014;103(4):461-472.
  21. Evans JL, Goldfine ID, Maddux BA, Grodsky GM. Oxidative stress and stress-activated signaling pathways: a unifying hypothesis of type 2 diabetes. Endocrine Rev. 2002;3(5):599-622.
  22. Gibson CM, Giugliano RP, Kloner RA, et al. EMBRACE STEMI study: a Phase 2a trial to evaluate the safety, tolerability, and efficacy of intravenous MTP-131 on reperfusion injury in patients undergoing primary percutaneous coronary intervention. Eur Heart J. 2016;37(16):1296-1303.
  23. Huang J, Li X, Li M, et al. Mitochondria-targeted antioxidant peptide SS31 protects the retinas of diabetic rats. Curr Mol Med. 2013;13(6):935-945.
  24. Hultman E, Sjöholm H. Energy metabolism and contraction force of human skeletal muscle in situ during electrical stimulation. J Physiol. 1983;345:525-532.
  25. James AM, Murphy MP. How mitochondrial damage affects cell function. J Biomed Sci. 2002;9(5-6):475-487.
  26. Kloner RA, Hale SL, Dai W, et al. Reduction of ischemia/reperfusion injury with Bendavia, a mitochondria-targeting cytoprotective peptide. J Am Heart Assoc. 2012;1(3):e001644.
  27. Lei H, Ugurbil K, Chen W. Measurement of unidirectional Pi to ATP flux in human visual cortex at 7 T by using in vivo 31P magnetic resonance spectroscopy. Proc Natl Acad Sci U S A. 2003;100(24):14409-14414.
  28. LHON 101. Available at: Accessed April 22, 2016.
  29. Li J, Chen X, Xiao W, et al. Mitochondria-targeted antioxidant peptide SS31 attenuates high glucose-induced injury on human retinal endothelial cells. Biochem Biophys Res Commun. 2011;404(1):349-356.
  30. Manfredi AA, Rovere-Querini P. The mitochondrion — a Trojan horse that kicks off inflammation? N Engl J Med. 2010;362(22):2132-2134.
  31. Muscular Dystrophy Association (MDA). Mitochondrial myopathies. Accessed April 22, 2016.
  32. National Institutes of Health National Eye Institute. Facts about diabetic retinopathy. Accessed July 22, 2014.
  33. National Institutes of Health. Leber hereditary optic neuropathy. Accessed April 22, 2016.
  34. Neubauer S. The failing heart—an engine out of fuel. N Engl J Med. 2007;356(11):1140-1151.
  35. Nishikawa T, Edelstein D, Du XL, et al. Normalizing mitochondrial superoxide production blocks three pathways of hyperglycaemic damage. Nature. 2000;404(6779):787-790.
  36. Okawa H, Sampath AP, Laughlin SB, Fain GL. ATP consumption by mammalian rod photoreceptors in darkness and in light. Curr Biol. 2008;18(24):1917-1921.
  37. Pfeffer G, Chinnery PF. Diagnosis and treatment of mitochondrial myopathies. Ann Med. 2013;45(1):4-16.
  38. Ross B, Freeman D, Chan L. Contributions of nuclear magnetic resonance to renal biochemistry. Kidney Int. 1986;29(1):131-141.
  39. Sabbah HN, Gupta RC, Kohli S, Wang M, Hachem S, Zhang K. Chronic therapy with elamipretide (MTP-131), a novel mitochondria-targeting peptide, improves left ventricular and mitochondrial function in dogs with advanced heart failure. Circ Heart Fail. 2016;9(2):e002206.
  40. Siegel MP, Kruse SE, Percival JM, et al. Mitochondrial-targeted peptide rapidly improves mitochondrial energetics and skeletal muscle performance in aged mice. Aging Cell. 2013;12(5):763-771.
  41. Sies H. Strategies of antioxidant defense. Eur J Biochem. 1993;215(2):213-219.
  42. Sloan RC, Moukdar F, Frasier CR, et al. Mitochondrial permeability transition in the diabetic heart: contributions of thiol redox state and mitochondrial calcium to augmented reperfusion injury. J Mol Cell Cardiol. 2012;52(5):1009-1018.
  43. Stanley WC, Recchia FA, Lopaschuk GD. Myocardial substrate metabolism in the normal and failing heart. Physiol Rev. 2005;85(3):1093-1129.
  44. Weinberg JM. Mitochondrial biogenesis in kidney disease. J Am Soc Nephrol. 2011;22(3):431-436.
  45. Werkmeister RM, Schmidl D, Aschinger G, et al. Retinal oxygen extraction in humans. Sci Rep. 2015;5:15763.
  46. Wong-Riley MT. Energy metabolism of the visual system. Eye Brain. 2010;2:99-116.
  47. Yau JW, Rogers SL, Kawasaki R, Lamoureux EL, Kowalski JW, Bek T, et al; Meta-Analysis for Eye Disease (META-EYE) Study Group. Global prevalence and major risk factors of diabetic retinopathy. Diabetes Care. 2012 Mar; 35 (3):556-64.
  48. Yu-Wai-Mana P, Griffiths PG, Chinnery PF. Mitochondrial optic neuropathies – disease mechanisms and therapeutic strategies. Prog Retin Eye Res. 2011;30(2):81-114.

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