A Structural Perspective on Respiratory Complex I

Structure and Function of NADH:ubiquinone oxidoreductase

  • Leonid Sazanov

Table of contents

  1. Front Matter
    Pages i-x
  2. Structure and Mechanism of Complex I

    1. Front Matter
      Pages 1-1
    2. Rouslan G. Efremov, Leonid Sazanov
      Pages 3-21
    3. Thorsten Friedrich, Petra Hellwig, Oliver Einsle
      Pages 23-59
    4. Eiko Nakamaru-Ogiso
      Pages 61-79
  3. Evolution of Complex I

    1. Front Matter
      Pages 107-107
    2. Anne Volbeda, Juan C. Fontecilla-Camps
      Pages 109-121
    3. Vamsi Krishna Moparthi, Cecilia Hägerhäll
      Pages 123-143
  4. Mutations in Complex I Subunits and Medical Implications

    1. Front Matter
      Pages 145-145
    2. Takao Yagi, Jesus Torres-Bacete, Prem Kumar Sinha, Norma Castro-Guerrero, Akemi Matsuno-Yagi
      Pages 147-169
    3. Mina Pellegrini, Jan A. M. Smeitink, Peter H. G. M. Willems, Werner J. H. Koopman
      Pages 171-189
  5. Subunit Composition and Assembly of Mitochondrial Complex I

    1. Front Matter
      Pages 191-191
    2. Jessica Nouws, Maria Antonietta Calvaruso, Leo Nijtmans
      Pages 193-217
    3. Claire Remacle, Patrice Hamel, Véronique Larosa, Nitya Subrahmanian, Pierre Cardol
      Pages 219-244
  6. Supercomplexes in Mitochondria

    1. Front Matter
      Pages 245-245
  7. Back Matter
    Pages 279-286

About this book


Complex I (NADH:ubiquinone oxidoreductase) is the first and largest enzyme of the respiratory chain in mitochondria and plays a central role in cellular energy production. Complex I dysfunction has been implicated in many human neurodegenerative diseases, including Parkinson’s.

Mutations in complex I subunits are among the most common human genetic disorders known. Until recently, in the absence of structural information, complex I was the least understood enzyme of the respiratory chain. The mechanism by which it couples electron transfer between NADH and quinone to the translocation of protons across the membrane was a complete enigma.

Recent years have been marked by spectacular progress in the elucidation of complex I’s structure, finally allowing the interpretation of a wealth of data accumulated in about 50 years since the discovery of the enzyme. Understanding of the mechanism of this large molecular machine, involving long-range conformational changes, is beginning to emerge.

This book is the first devoted entirely to complex I. It contains chapters written by leaders in the field, covering a wide range of topics from the structure of the complex, properties of its many redox centers, subunit composition, mutagenesis studies, evolution of the enzyme and on to the current understanding of the coupling mechanism and the molecular basis for human pathologies.


- Concise and authoritative review of current state-of-the-art in research on respiratory complex I

- The first book entirely devoted to complex I, a central enzyme in bioenergetics

- Recent breakthroughs in structure elucidation finally allow understanding of the mechanism

- Mutations leading to common and severe human disorders are discussed.


NADH:ubiquinone oxidoreductase (complex I) bioenergetics electron transfer and proton translocation membrane protein structure respiratory chain

Editors and affiliations

  • Leonid Sazanov
    • 1
  1. 1., Mitochondrial Biology UnitMedical Research CouncilCambridgeUnited Kingdom

Bibliographic information