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Nucleic Acids Research Advance Access originally published online on February 18, 2007
Nucleic Acids Research 2007 35(5):1638-1648; doi:10.1093/nar/gkm033
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Nucleic Acids Research, 2007, Vol. 35, No. 5 1638-1648
© 2007 The Author(s).
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.0/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Structural Biology

Insights into Fanconi Anaemia from the structure of human FANCE

Ravi K. Nookala, Shobbir Hussain and Luca Pellegrini*

Department of Biochemistry, University of Cambridge, Tennis Court Road, Cambridge, CB2 1GA, UK

*To whom correspondence should be addressed. Tel: 00441223333667; Fax: 00441223766002; Email: luca{at}cryst.bioc.cam.ac.uk

Received November 2, 2006. Revised December 20, 2006. Accepted January 5, 2007.

Fanconi Anaemia (FA) is a cancer predisposition disorder characterized by spontaneous chromosome breakage and high cellular sensitivity to genotoxic agents. In response to DNA damage, a multi-subunit assembly of FA proteins, the FA core complex, monoubiquitinates the downstream FANCD2 protein. The FANCE protein plays an essential role in the FA process of DNA repair as the FANCD2-binding component of the FA core complex. Here we report a crystallographic and biological study of human FANCE. The first structure of a FA protein reveals the presence of a repeated helical motif that provides a template for the structural rationalization of other proteins defective in Fanconi Anaemia. The portion of FANCE defined by our crystallographic analysis is sufficient for interaction with FANCD2, yielding structural information into the mode of FANCD2 recruitment to the FA core complex. Disease-associated mutations disrupt the FANCE–FANCD2 interaction, providing structural insight into the molecular mechanisms of FA pathogenesis.


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