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ACCEPTED MANUSCRIPT<br />

Annual review of biochemistry 76 (2007) 723-749.<br />

[90] S. Dimauro, G. Davidzon, Mitochondrial DNA and disease, Annals of<br />

medicine 37 (2005) 222-232.<br />

[91] R. Rossignol, B. Faustin, C. Rocher, M. Malgat, J.P. Mazat, T. Letellier,<br />

Mitochondrial threshold effects, The Biochemical journal 370 (2003) 751-762.<br />

[92] A.H. Schapira, Mitochondrial disease, Lancet 368 (2006) 70-82.<br />

[93] Y. Kraytsberg, E. Kudryavtseva, A.C. McKee, C. Geula, N.W. Kowall, K.<br />

Khrapko, Mitochondrial DNA deletions are abundant and cause functional<br />

impairment in aged human substantia nigra neurons, Nature genetics 38<br />

(2006) 518-520.<br />

[94] A. Bender, K.J. Krishnan, C.M. Morris, G.A. Taylor, A.K. Reeve, R.H. Perry,<br />

E. Jaros, J.S. Hersheson, J. Betts, T. Klopstock, R.W. Taylor, D.M. Turnbull,<br />

High levels of mitochondrial DNA deletions in substantia nigra neurons in<br />

aging and Parkinson disease, Nature genetics 38 (2006) 515-517.<br />

[95] A.K. Reeve, K.J. Krishnan, J.L. Elson, C.M. Morris, A. Bender, R.N.<br />

Lightowlers, D.M. Turnbull, Nature of mitochondrial DNA deletions in<br />

substantia nigra neurons, American journal of human genetics 82 (2008) 228-<br />

235.<br />

ACCEPTED MANUSCRIPT<br />

[96] P. Luoma, A. Melberg, J.O. Rinne, J.A. Kaukonen, N.N. Nupponen, R.M.<br />

Chalmers, A. Oldfors, I. Rautakorpi, L. Peltonen, K. Majamaa, H. Somer, A.<br />

Suomalainen, Parkinsonism, premature menopause, and mitochondrial DNA<br />

polymerase gamma mutations: clinical and molecular genetic study, Lancet<br />

364 (2004) 875-882.<br />

[97] P.T. Luoma, J. Eerola, S. Ahola, A.H. Hakonen, O. Hellstrom, K.T. Kivisto,<br />

P.J. Tienari, A. Suomalainen, Mitochondrial DNA polymerase gamma<br />

51

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