Int. J. Mol. Sci. 2013, 14(2), 2916-2927; doi:10.3390/ijms14022916
Review

Oxidative Folding in the Mitochondrial Intermembrane Space in Human Health and Disease

1,* email and 1,2,* email
1 Department of Biochemistry and Molecular Biology, Autonomous University of Barcelona, Bellaterra E-08193, Spain 2 Institute of Biotechnology and Biomedicine, Autonomous University of Barcelona, Bellaterra E-08193, Spain
* Authors to whom correspondence should be addressed.
Received: 21 December 2012; in revised form: 21 January 2013 / Accepted: 23 January 2013 / Published: 30 January 2013
(This article belongs to the Special Issue Redox Signaling in Biology and Patho-Biology)
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Abstract: Oxidative folding in the mitochondrial intermembrane space (IMS) is a key cellular event associated with the folding and import of a large and still undetermined number of proteins. This process is catalyzed by an oxidoreductase, Mia40 that is able to recognize substrates with apparently little or no homology. Following substrate oxidation, Mia40 is reduced and must be reoxidized by Erv1/Alr1 that consequently transfers the electrons to the mitochondrial respiratory chain. Although our understanding of the physiological relevance of this process is still limited, an increasing number of pathologies are being associated with the impairment of this pathway; especially because oxidative folding is fundamental for several of the proteins involved in defense against oxidative stress. Here we review these aspects and discuss recent findings suggesting that oxidative folding in the IMS is modulated by the redox state of the cell.
Keywords: cysteine motifs; disulfide bonds; Erv1; intermembrane space; Mia40; mitochondria; oxidative protein folding; protein import; oxidative stress

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MDPI and ACS Style

Fraga, H.; Ventura, S. Oxidative Folding in the Mitochondrial Intermembrane Space in Human Health and Disease. Int. J. Mol. Sci. 2013, 14, 2916-2927.

AMA Style

Fraga H, Ventura S. Oxidative Folding in the Mitochondrial Intermembrane Space in Human Health and Disease. International Journal of Molecular Sciences. 2013; 14(2):2916-2927.

Chicago/Turabian Style

Fraga, Hugo; Ventura, Salvador. 2013. "Oxidative Folding in the Mitochondrial Intermembrane Space in Human Health and Disease." Int. J. Mol. Sci. 14, no. 2: 2916-2927.

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