Journal of IiME Volume 1 Issue 2 www.investinme.org Gene therapy for mitochondrial dysfunctions using optimized mRNA transport to the mitochondrial surface (continued) 26. Margeot A, Blugeon C, J. Sylvestre, Jacq C, CorralDebrinski M. In Saccharomyces cerevisiae, ATP2 mRNA sorting to the vicinity of mitochondria is essential for respiratory function. EMBO J. 2002;21(24):6893-6904. 27. Sylvestre J, Margeot A, Jacq C, Dujardin G, CorralDebrinski M. The role of the 3'UTR in mRNA sorting to the vicinity of mitochondria is conserved from yeast to human cells. Mol. Biol. Cell 2003;14:3848-3856. 28. Corral-Debrinski M, Blugeon C, Jacq C. In yeast, the 3' Untranslated Region or the presequence of ATM1 is required for the exclusive localization of its mRNA to the vicinity of mitochondria. Mol. Cell Biol. 2000;20(21):78817892. 29. Ginsberg MD, Feliciello A, Jones JK, Avvedimento EV, Gottesman ME. PKA-dependent binding of mRNA to the mitochondrial AKAP121 protein. J. Mol. Biol. 2003;327(4):885-897. 30. Kaltimbacher V, C.Bonnet, Lecoeuvre G, Forster V, Sahel J-A, Corral-Debrinski M. mRNA localization to the mitochondrial surface allows the efficient translocation inside the organelle of a nuclear recoded ATP6 protein. RNA 2006;12(7):1408-1417. 31. Benit P., Goncalves S., Dassa E. P., Briere J.J., Martin G., Rustin. Three spectrophotometric assays for the measurement of the five respiratory chain complexes in minuscule biological samples. Clin Chim Acta. 2006;374(1-2):81-86. 32. Bonnet C, Kaltimbacher V, Ellouze S, et al. Allotopic mRNA localization to the mitochondrial surface rescues respiratory chain defects in fibroblasts harboring mtDNA mutations affecting complex I or V subunits. Rejuvenation Res. 2007;10:128-144. 33. Kasahara A IK, Yamaoka M, Ito M, Watanabe N, Akimoto M, Sato A, Nakada K, Endo H, Suda Y, Aizawa S, Hayashi J. Generation of trans-mitochondrial mice carrying homoplasmic mtDNAs with a missense mutation in a structural gene using ES cells. Hum Mol Genet 2006;15(6):871-881. 34. Ishikawa H TM, Matsumoto N, Sawada H, Ide C, Mimura O, Dezawa M. Effect of GDNF gene transfer into axotomized retinal ganglion cells using in vivo electroporation with a contact lens-type electrode. Gene Ther.2005;12(4):289-298. 35. Tezel G. Oxidative stress in glaucomatous neurodegeneration: mechanisms and consequences. Prog. Retin. Eye Res. 2006;25:490-513. 36. Züchner S MI, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J, Parman Y, Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battaloglu E, Polyakov AV, Timmerman V, Schröder JM, Vance JM. Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat. Genet. 2004;36:449-451. 37. Qi X SL, Lewin AS, Hauswirth WW, Guy J. The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse. Invest Ophthalmol Vis Sci 2007;48:1-10. ME Story In a few weeks time it will be the one year anniversary of my gorgeous and funny and talented sister's death. If you knew her before she got ill she was like a force of nature. Talented, funny, generous she had loads of friends and was very much a person who lived her life to the full. She had courage and was original and so much more. M.E. was the cruellest thing to ever happen to Sophia. I will not go on about how much she suffered because it is an unbelievable amount. To top it all her illness was not recognised as a neurological disease and so there was the added burden of trying to get the authorities to understand the true nature of her illness. Unfortunately for us all Sophia suffered even more than was necessary. My amazing sister has paid with her life but she all she wanted was that if only one person was helped by her experience it would all have been worth it for her. From Sophia Mirza's sister - Roisin Mirza (written in 2006) Invest in ME Charity Nr 1114035 Page 29/72

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