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  • The EMBO Journal (2007) 26, 4347 - 4358
  • doi:10.1038/sj.emboj.7601862

Published online: 20 September 2007

Shy1 couples Cox1 translational regulation to cytochrome c oxidase assembly

David U Mick1,2, Karina Wagner1,2, Martin van der Laan1, Ann E Frazier1,3, Inge Perschil1, Magdalena Pawlas4, Helmut E Meyer4, Bettina Warscheid4 and Peter Rehling1

  1. Institut für Biochemie und Molekularbiologie, Zentrum für Biochemie und Molekulare Zellforschung, Universität Freiburg, Freiburg, Germany
  2. Fakultät für Biologie, Universität Freiburg, Freiburg, Germany
  3. Department of Biochemistry, La Trobe University, Melbourne, Australia
  4. Medizinisches Proteom-Center, Ruhr-Universität Bochum, Bochum, Germany

Correspondence to:

Peter Rehling, Institut für Biochemie und Molekularbiologie, Zentrum für Biochemie und Molekulare Zellforschung, Universität Freiburg, Hermann-Herder-Stras zlige 7, Freiburg 79104, Germany. Tel.: +49 761 203 5245; Fax: +49 761 203 5245; E-mail: peter.rehling@biochemie.uni-freiburg.de

Received 20 April 2007; Accepted 24 August 2007

Cytochrome c oxidase (complex IV) of the respiratory chain is assembled from nuclear and mitochondrially-encoded subunits. Defects in the assembly process lead to severe human disorders such as Leigh syndrome. Shy1 is an assembly factor for complex IV in Saccharomyces cerevisiae and mutations of its human homolog, SURF1, are the most frequent cause for Leigh syndrome. We report that Shy1 promotes complex IV biogenesis through association with different protein modules; Shy1 interacts with Mss51 and Cox14, translational regulators of Cox1. Additionally, Shy1 associates with the subcomplexes of complex IV that are potential assembly intermediates. Formation of these subcomplexes depends on Coa1 (YIL157c), a novel assembly factor that cooperates with Shy1. Moreover, partially assembled forms of complex IV bound to Shy1 and Cox14 can associate with the bc1 complex to form transitional supercomplexes. We suggest that Shy1 links Cox1 translational regulation to complex IV assembly and supercomplex formation.

  • Keywords:

    • Leigh syndrome,
    • mitochondria,
    • protein complex assembly,
    • respiratory chain complex,
    • SURF1