Selected recent peer reviewed publications (from a total of 42)

25. D'Aurelio M, Pallotti F, Barrientos A, Gajewski CD, Kwong JQ, Bruno C, Beal MF, Manfredi G*. (2001) In Vivo Regulation of Oxidative Phosphorylation in Cells Harboring a Stop-codon Mutation in Mitochondrial DNA-encoded Cytochrome c Oxidase Subunit I. J Biol Chem; 276:46925-46932.

26. Manfredi G., Fu J., Ojaimi J., Sadlock J. E., Kwong J. Q., Guy J., and Schon E. A. (2002) Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus. Nat Genet. 30, 394-399.

27. Vives-Bauza C., Andreu A. L., Manfredi G., Beal M. F., Janetzky B., Gruenewald T. H., and Lin M. T. (2002) Sequence analysis of the entire mitochondrial genome in Parkinson's disease. Biochem Biophys Res Commun. 290, 1593-1601.

28. Wiedemann F. R., Manfredi G., Mawrin C., Beal M. F., and Schon E. A. (2002) Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients. J Neurochem. 80, 616-625.

29. Mattiazzi M, D'Aurelio M, Gajewski CD, Martushova K, Kiaei M, Beal MF, Manfredi G*. (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem 277:29626-33.

30. Chirichigno, J.W., Manfredi, G., Beal, M.F. and Albers, D.S. (2002) Stress-induced mitochondrial depolarization and oxidative damage in PSP cybrids. Brain Res., 951:31-35.

31. Guy, J., Xiaoping, Q., Pallotti, F., Schon, E.A., Manfredi, G., Carelli, V., Martinuzzi, A., Hauswirth, W.W., Lewin, A.S. (2002) Rescue of a mitochondrial deficiency causing Leber hereditary optic neuropathy. Ann Neurol. 52:534-542.

32. Gajewski CD, Lin MT, Cudkowicz ME, Beal MF, Manfredi G* (2003) Mitochondrial DNA from platelets of sporadic ALS patients restores normal respiratory functions in rho(0) cells. Exp Neurol 179:229-35

33. Manfredi G*, Kwong JQ, Oca-Cossio JA, Woischnik M, Gajewski CD, Martushova K, D'Aurelio M, Friedlich AL, Moraes CT (2003) BCL-2 improves oxidative phosphorylation and modulates adenine nucleotide translocation in mitochondria of cells harboring mutant mtDNA. J Biol Chem 278:5639-45

34. Barrientos, A., Korr, D., Barwell, K.J., Sjulsen, C., Gajewski, C.D., Manfredi, G., Ackerman, S. and Tzagoloff, A. (2003) MTG1 codes for a conserved protein required for mitochondrial translation. Mol Biol Cell, 14, 2292-2302.

35. Bayona-Bafaluy MP, Manfredi G, Moraes CT (2003) A chemical enucleation method for the transfer of mitochondrial DNA to rho(o) cells. Nucleic Acids Res 31:e98

36. Gajewski CD, Yang L, Schon EA, Manfredi G* (2003) New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters. Mol Biol Cell 14:3628-35

37. Mattiazzi, M., Vijayvergiya, C., Gajewski, C. D., DeVivo, D. C., Lenaz, G., Wiedmann, M., Manfredi G*. (2004). The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Hum Mol Genet. 15;13(8):869-79.

38. Manfredi G, Xu Z. Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion 2005;5(2):77-87.

39. Vijayvergiya C, Beal MF, Buck J, Manfredi G*. Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci 2005;25(10):2463-2470.

40. Shidara Y, Yamagata K, Kanamori T, Nakano K, Kwong JQ, Manfredi G, Oda H, Ohta S. Positive contribution of pathogenic mutations in the mitochondrial genome to the promotion of cancer by prevention from apoptosis. Cancer Res 2005;65(5):1655-1663.

41. Hervias S, Beal MF, Manfredi G*, Mitochondrial dysfunction in amyotrophic lateral sclerosis. Muscle Nerve 2005 (in press).

42. Damiano M, Starkov AA, Petri A, Kipiani K, Kiaei K, Mattiazzi M, Beal MF, Manfredi G*. Neural Mitochondrial Ca2+ Capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-Superoxide Dismutase Mutant Mice. J Neurochem 2005 (in press).