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

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.

Bibliographic Details
Main Authors: 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
Format: Article
Language:English
Published: Nature Publishing Group 2019-05-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-019-09989-y