Oxidative folding in the mitochondrial intermembrane space in human health and disease
Fraga, Hugo (Universitat Autònoma de Barcelona. Departament de Bioquímica i de Biologia Molecular)
Ventura, Salvador (Universitat Autònoma de Barcelona. Institut de Biotecnologia i de Biomedicina "Vicent Villar Palasí")

Date:	2013
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.
Grants:	Ministerio de Ciencia e Innovación BFU2010-14901 Agència de Gestió d'Ajuts Universitaris i de Recerca 2009/SGR-760 Agència de Gestió d'Ajuts Universitaris i de Recerca 2009/CTP-00004
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Cysteine motifs ; Disulfide bonds ; Erv1 ; Intermembrane space ; Mia40 ; Mitochondria ; Oxidative protein folding ; Protein import ; Oxidative stress
Published in:	International journal of molecular sciences, Vol. 14, issue 2 (Jan. 2013) , p. 2916-2927, ISSN 1422-0067

DOI: 10.3390/ijms14022916
PMID: 23364613

12 p, 278.9 KB

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Research literature > UAB research groups literature > Research Centres and Groups (research output) > Health sciences and biosciences > Institut de Biotecnologia i de Biomedicina (IBB)
Articles > Research articles
Articles > Published articles