Crystal structure of the entire respiratory complex I

Nature. 2013 Feb 28;494(7438):443-8. doi: 10.1038/nature11871. Epub 2013 Feb 17.

Abstract

Complex I is the first and largest enzyme of the respiratory chain and has a central role in cellular energy production through the coupling of NADH:ubiquinone electron transfer to proton translocation. It is also implicated in many common human neurodegenerative diseases. Here, we report the first crystal structure of the entire, intact complex I (from Thermus thermophilus) at 3.3 Å resolution. The structure of the 536-kDa complex comprises 16 different subunits, with a total of 64 transmembrane helices and 9 iron-sulphur clusters. The core fold of subunit Nqo8 (ND1 in humans) is, unexpectedly, similar to a half-channel of the antiporter-like subunits. Small subunits nearby form a linked second half-channel, which completes the fourth proton-translocation pathway (present in addition to the channels in three antiporter-like subunits). The quinone-binding site is unusually long, narrow and enclosed. The quinone headgroup binds at the deep end of this chamber, near iron-sulphur cluster N2. Notably, the chamber is linked to the fourth channel by a 'funnel' of charged residues. The link continues over the entire membrane domain as a flexible central axis of charged and polar residues, and probably has a leading role in the propagation of conformational changes, aided by coupling elements. The structure suggests that a unique, out-of-the-membrane quinone-reaction chamber enables the redox energy to drive concerted long-range conformational changes in the four antiporter-like domains, resulting in translocation of four protons per cycle.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Benzoquinones / chemistry
  • Benzoquinones / metabolism
  • Cell Membrane / metabolism
  • Crystallography, X-Ray
  • Electron Transport Complex I / chemistry*
  • Electron Transport Complex I / metabolism*
  • Humans
  • Hydrophobic and Hydrophilic Interactions
  • Models, Molecular
  • NAD / metabolism
  • Oxidation-Reduction
  • Protein Folding
  • Protein Subunits / chemistry
  • Protein Subunits / metabolism
  • Proton-Motive Force
  • Protons
  • Thermus thermophilus / chemistry*
  • Thermus thermophilus / cytology
  • Ubiquinone / metabolism

Substances

  • Benzoquinones
  • Protein Subunits
  • Protons
  • NAD
  • Ubiquinone
  • quinone
  • Electron Transport Complex I

Associated data

  • PDB/4HE8
  • PDB/4HEA