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Originally published In Press as doi:10.1074/jbc.M203065200 on June 5, 2002

J. Biol. Chem., Vol. 277, Issue 33, 29626-29633, August 16, 2002
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Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice*

Marina MattiazziDagger , Marilena D'AurelioDagger , Carl D. Gajewski, Katherine Martushova, Mahmoud Kiaei, M. Flint Beal, and Giovanni Manfredi§

From the Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021

A growing body of evidence suggests that impaired mitochondrial energy production and increased oxidative radical damage to the mitochondria could be causally involved in motor neuron death in amyotrophic lateral sclerosis (ALS) and in familial ALS associated with mutations of Cu,Zn superoxide dismutase (SOD1). For example, morphologically abnormal mitochondria and impaired mitochondrial histoenzymatic respiratory chain activities have been described in motor neurons of patients with sporadic ALS. To investigate further the role of mitochondrial alterations in the pathogenesis of ALS, we studied mitochondria from transgenic mice expressing wild type and G93A mutated hSOD1. We found that a significant proportion of enzymatically active SOD1 was localized in the intermembrane space of mitochondria. Mitochondrial respiration, electron transfer chain, and ATP synthesis were severely defective in G93A mice at the time of onset of the disease. We also found evidence of oxidative damage to mitochondrial proteins and lipids. On the other hand, presymptomatic G93A transgenic mice and mice expressing the wild type form of hSOD1 did not show significant mitochondrial abnormalities. Our findings suggest that G93A-mutated hSOD1 in mitochondria may cause mitochondrial defects, which contribute to precipitating the neurodegenerative process in motor neurons.

* This work was supported by grants from the New York Academy of Medicine "Speaker's Fund" (to M. D'A., C. D. G., and G. M.), the ALS Association (to M. F. B.), and National Institutes of Health Grant PO1-AG12992 (to M. F. B.).The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Dagger Both authors contributed equally to this work.

§ To whom correspondence should be addressed: Weill Medical College of Cornell University, 525 E. 68th St., A-505, New York, NY 10021. Tel.: 212-746-4605; Fax: 212-746-4803; E-mail: gim2004@mail.med.cornell.edu.

Copyright © 2002 by The American Society for Biochemistry and Molecular Biology, Inc.
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