Protein & Cell

, Volume 2, Issue 9, pp 704–711

The Fanconi anemia pathway and DNA interstrand cross-link repair

Review

DOI: 10.1007/s13238-011-1098-y

Cite this article as:
Su, X. & Huang, J. Protein Cell (2011) 2: 704. doi:10.1007/s13238-011-1098-y

Abstract

Fanconi anemia (FA) is an autosomal or X-linked recessive disorder characterized by chromosomal instability, bone marrow failure, cancer susceptibility, and a profound sensitivity to agents that produce DNA interstrand cross-link (ICL). To date, 15 genes have been identified that, when mutated, result in FA or an FA-like syndrome. It is believed that cellular resistance to DNA interstrand cross-linking agents requires all 15 FA or FA-like proteins. Here, we review our current understanding of how these FA proteins participate in ICL repair and discuss the molecular mechanisms that regulate the FA pathway to maintain genome stability.

Keywords

Fanconi anemia DNA interstrand crosslink repair FANCD2-FANCI mono-ubiquitylation chromosomal instability 

Copyright information

© Higher Education Press and Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  1. 1.Life Sciences InstituteZhejiang UniversityHangzhouChina

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