Nature Communications (May 2019)

Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism

  • Nicholas G. Fox,
  • Xiaodi Yu,
  • Xidong Feng,
  • Henry J. Bailey,
  • Alain Martelli,
  • Joseph F. Nabhan,
  • Claire Strain-Damerell,
  • Christine Bulawa,
  • Wyatt W. Yue,
  • Seungil Han

DOI
https://doi.org/10.1038/s41467-019-09989-y
Journal volume & issue
Vol. 10, no. 1
pp. 1 – 8

Abstract

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The iron-sulfur cluster (ISC) assembly complex is activated by frataxin (FXN) and Friedreich’s ataxia is caused by FXN deficiency. Here the authors present the 3.2 Å resolution cryo-EM structure of the human frataxin bound ISC complex and discuss how FXN activates enzymatic activity.