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J. Biol. Chem., Vol. 276, Issue 41, 38084-38089, October 12, 2001
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From the Cu,Zn-superoxide dismutase (SOD1) is an
abundant, largely cytosolic enzyme that scavenges superoxide anions.
The biological role of SOD1 is somewhat controversial because
superoxide is thought to arise largely from the mitochondria where a
second SOD (manganese SOD) already resides. Using bakers' yeast as a
model, we demonstrate that Cu,Zn-SOD1 helps protect mitochondria from
oxidative damage, as sod1
A Fraction of Yeast Cu,Zn-Superoxide Dismutase and Its
Metallochaperone, CCS, Localize to the Intermembrane Space of
Mitochondria
A PHYSIOLOGICAL ROLE FOR SOD1 IN GUARDING AGAINST MITOCHONDRIAL
OXIDATIVE DAMAGE*
§,,
Department of Environmental Health Sciences,
Johns Hopkins University Bloomberg School of Public Health, Baltimore,
Maryland 21205 and the ¶ Institut fur Zytobiologie der
Philipps-Universitat Marburg, Robert-Koch-Str. 5, 35033 Marburg,
Germany
mutants show elevated protein
carbonyls in this organelle. In accordance with this connection to
mitochondria, a fraction of active SOD1 localizes within the
intermembrane space (IMS) of mitochondria together with its copper
chaperone, CCS. Neither CCS nor SOD1 contains typical N-terminal
presequences for mitochondrial uptake; however, the mitochondrial
accumulation of SOD1 is strongly influenced by CCS. When CCS synthesis
is repressed, mitochondrial SOD1 is of low abundance, and conversely
IMS SOD1 is very high when CCS is largely mitochondrial. The
mitochondrial form of SOD1 is indeed protective against oxidative
damage because yeast cells enriched for IMS SOD1 exhibit prolonged
survival in the stationary phase, an established marker of
mitochondrial oxidative stress. Cu,Zn-SOD1 in the mitochondria appears
important for reactive oxygen physiology and may have critical
implications for SOD1 mutations linked to the fatal neurodegenerative
disorder, amyotrophic lateral sclerosis.
*
This work was supported in part by the Johns Hopkins
University NIEHS center, by National Institutes of Health Grant GM50016 (to V. C.), and by funding from Sonderforschungsbereich 286 of the Deutsche Forschungsgemeinschaft, the Volkswagen-Stiftung, and Chemischen Industrie (to R. L.).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.
To whom correspondence should be addressed: Johns Hopkins
University, 615 N. Wolfe Street, Room 7032, Baltimore, MD 21205. Tel.:
410-955-3029; Fax: 410-955-0116; E-mail: vculotta@jhsph.edu.
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