Our Science: References

  1. Daiger SP, Sullivan LS, Bowne SJ. Genes and mutations causing retinitis pigmentosa. Clin Genet. 2013;84(2):132‐141.
  2. "Usher Syndrome", NIDCD website
  3. Trademarks are the property of their owners.
  4. Shen J, Yang X, Dong A, Petters RM, Peng YW, Wong F, Campochiaro PA. Oxidative damage is a potential cause of cone cell death in retinitis pigmentosa. J Cell Physiol. 2005 Jun;203(3):457-64.
  5. Komeima K, Rogers BS, Lu L, Campochiaro PA. Antioxidants reduce cone cell death in a model of retinitis pigmentosa. Proc Natl Acad Sci USA 2006; 103: 11300-11305.
  6. Komeima K, Rogers BS, Campochiaro PA. Antioxidants slow photoreceptor cell death in mouse models of retinitis pigmentosa. J Cell Physiol 2007; 213:809-815.
  7. Tuson M, Garanto A, Gonzalez-Duarte R, Marfany G. Over-expression of CERKL, a gene responsible for retinitis pigmentosa in humans, protects cells from apoptosis induced by oxidative stress. Molec Vis [serial online] 2009; 15:168-180.
  8. Usui S, Komeima K, Lee SY, et al. Increased expression of catalase and superoxide dismutase 2 reduces cone cell death in retinitis pigmentosa. Mol Ther 2009a; 17(5):778-786.
  9. Usui S, Overson BC, Lee SY, et al. NADPH oxidase plays a central role in cone cell death in retinitis pigmentosa. J Neurochem 2009b; 110: 1028-1037.
  10. Campochiaro PA, Mir TA. The mechanism of cone cell death in Retinitis Pigmentosa. Prog Retin Eye Res. 2018 Jan;62:24-37.
  11. Campochiaro PA, Strauss R, Lu L, Hafiz G, Wolfson Y, Shah SM, Sophie R, Mir TA and Scholl HP. Is there excess oxidative stress and damage in eyes of patients with retinitis pigmentosa? Antioxid Redox Signal. 2015 Sep 1; 23(7): 643–648.
  12. Sunitha K, Hemshekhar M, Thushara RM, Santhosh MS, Yariswamy M, Kemparaju K, Girish KS. N-Acetylcysteine amide: a derivative to fulfill the promises of N-Acetylcysteine. Free Radic Res. 2013 May;47(5):357-67.
  13. Lee SY, Usui S, Zafar A-B, Oveson BD, Jo, Y-J, Lu L, Masoudi S, Campochiaro PA. N-AcetyIcysteine promotes long-term survival of cones in a model of retinitis pigimentosa. J Cell Physiol 2011; 226; 1843-1849.
  14. Dong A, Stevens R, Hackett S, Campochiaro PA. Compared with N-acetylcysteine (NAC), N-Acetylcysteine Amide (NACA) Provides Increased Protection of Cone Function in a Model of Retinitis Pigmentosa. Investigative Ophthalmology & Visual Science April 2014, Vol.55, 1736.
  15. Campochiaro PA, Iftikhar M, Hafiz G, Akhlaq A, Tsai G, Wehling D, Lu L, Wall GM, Singh MS, Kong X. Oral N-acetylcysteine improves cone function in retinitis pigmentosa patients in phase I trial. J Clin Invest. 2020 Mar 2;130(3):1527-1541.
  16. Grinberg L, Fibach E, Amer J, Atlas D. N-acetylcysteine amide, a novel cell-permeating thiol, restores cellular glutathione and protects human red blood cells from oxidative stress.Free Radic Biol Med. 2005 Jan 1;38(1):136-45.
  17. Sunitha K, Hemshekhar M, Thushara RM, Santhosh MS, Yariswamy M, Kemparaju K, Girish KS. Free Radic Res. 2013 May;47(5):357-67.
  18. Babizhayev MA, Deyev AI, Yermakova VN, et al. Revival of the lens transparency with N-acetylcarnosine. Curr Drug Ther. 2006;1:91–116.
  19. Carey JW, Pinarci EY, Penugonda S, Karacal H, Ercal N. In vivo inhibition of L-buthionine-(S,R)-sulfoximine-induced cataracts by a novel antioxidant N-acetylcysteine amide. Free Radic Biol Med 2010;doi:10.1016/j.freeradbiomed.2010.12.017.
  20. Maddirala Y, Tobwala S, Karacal H, Ercal N. Prevention and reversal of selenite-induced cataracts by N-acetylcysteine amide in Wistar rats. BMC Ophthalmol. 2017 Apr 26;17(1):54.