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Cell Press, Cell Metabolism, 3(15), p. 324-335, 2012

DOI: 10.1016/j.cmet.2012.01.015

Elsevier, BBA - Bioenergetics, (1817), p. S140, 2012

DOI: 10.1016/j.bbabio.2012.06.369

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Mitochondrial complex I plays an essential role in human respirasome assembly

Journal article published in 2012 by David Moreno Lastres, Flavia Fontanesi, Inés García Consuegra, Miguel A. Martín, Joaquín Arenas ORCID, Antoni Barrientos, Cristina Ugalde ORCID
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This paper is available in a repository.

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Abstract

The biogenesis and function of the mitochondrial respiratory chain (RC) involve the organization of RC enzyme complexes in supercomplexes or respirasomes through an unknown biosynthetic process. This leads to structural interdependences between RC complexes, which are highly relevant from biological and biomedical perspectives, because RC defects often lead to severe neuromuscular disorders. We show that in human cells, respirasome biogenesis involves a complex I assembly intermediate acting as a scaffold for the combined incorporation of complexes III and IV subunits, rather than originating from the association of preassembled individual holoenzymes. The process ends with the incorporation of complex I NADH dehydrogenase catalytic module, which leads to the respirasome activation. While complexes III and IV assemble either as free holoenzymes or by incorporation of free subunits into supercomplexes, the respirasomes constitute the structural units where complex I is assembled and activated, thus explaining the significance of the respirasomes for RC function.