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.

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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:EN
Published: Nature Portfolio 2019
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Online Access:https://doaj.org/article/8abcf6459af9457bb7545657bbc6ed8d
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