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Anemia
Volume 2012 (2012), Article ID 425814, 11 pages
http://dx.doi.org/10.1155/2012/425814
Review Article

Fanconi Anemia Proteins and Their Interacting Partners: A Molecular Puzzle

1Department of Pediatrics, Université Laval, Cité Universitaire, Québec, QC, Canada G1K 7P4
2Reproduction, Perinatal Health and Child Health, Centre de Recherche du CHUQ-CHUL, 2705 Boul Laurier, Québec, QC, Canada G1V 4G2

Received 9 December 2011; Accepted 13 March 2012

Academic Editor: Henri J. van de Vrugt

Copyright © 2012 Tagrid Kaddar and Madeleine Carreau. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

In recent years, Fanconi anemia (FA) has been the subject of intense investigations, primarily in the DNA repair research field. Many discoveries have led to the notion of a canonical pathway, termed the FA pathway, where all FA proteins function sequentially in different protein complexes to repair DNA cross-link damages. Although a detailed architecture of this DNA cross-link repair pathway is emerging, the question of how a defective DNA cross-link repair process translates into the disease phenotype is unresolved. Other areas of research including oxidative metabolism, cell cycle progression, apoptosis, and transcriptional regulation have been studied in the context of FA, and some of these areas were investigated before the fervent enthusiasm in the DNA repair field. These other molecular mechanisms may also play an important role in the pathogenesis of this disease. In addition, several FA-interacting proteins have been identified with roles in these “other” nonrepair molecular functions. Thus, the goal of this paper is to revisit old ideas and to discuss protein-protein interactions related to other FA-related molecular functions to try to give the reader a wider perspective of the FA molecular puzzle.