Holding All the Cards-How Fanconi Anemia Proteins Deal with Replication Stress and Preserve Genomic Stability

Genes
Arindam Datta, Robert M Brosh

Abstract

Fanconi anemia (FA) is a hereditary chromosomal instability disorder often displaying congenital abnormalities and characterized by a predisposition to progressive bone marrow failure (BMF) and cancer. Over the last 25 years since the discovery of the first linkage of genetic mutations to FA, its molecular genetic landscape has expanded tremendously as it became apparent that FA is a disease characterized by a defect in a specific DNA repair pathway responsible for the correction of covalent cross-links between the two complementary strands of the DNA double helix. This pathway has become increasingly complex, with the discovery of now over 20 FA-linked genes implicated in interstrand cross-link (ICL) repair. Moreover, gene products known to be involved in double-strand break (DSB) repair, mismatch repair (MMR), and nucleotide excision repair (NER) play roles in the ICL response and repair of associated DNA damage. While ICL repair is predominantly coupled with DNA replication, it also can occur in non-replicating cells. DNA damage accumulation and hematopoietic stem cell failure are thought to contribute to the increased inflammation and oxidative stress prevalent in FA. Adding to its confounding nature, certain FA gene produc...Continue Reading

References

Jan 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·P F LinP Howard-Flanders
Apr 12, 1996·Science·C E YuG D Schellenberg
Jul 7, 2000·The Journal of Biological Chemistry·I KuraokaR D Wood
Mar 10, 2001·Molecular Cell·I Garcia-HigueraA D D'Andrea
Mar 10, 2001·Molecular Cell·M E MoynahanM Jasin
Feb 2, 2002·The EMBO Journal·Andrés Aguilera
Mar 9, 2002·Molecular and Cellular Biology·Nianxiang ZhangRandy J Legerski
Mar 22, 2002·The Lancet Oncology·P J McHughJ A Hartley
Jun 18, 2002·Science·Niall G HowlettAlan D D'Andrea
Sep 21, 2002·Molecular and Cellular Biology·Yannick SaintignyRaymond J Monnat
Oct 16, 2003·Molecular and Cellular Biology·Ulrica K WestermarkMary Ellen Moynahan
Apr 30, 2004·Human Molecular Genetics·Shobbir HussainChristopher G Mathew
Oct 16, 2004·Molecular and Cellular Biology·Jeremy M StarkMaria Jasin
Jan 15, 2005·Proceedings of the National Academy of Sciences of the United States of America·Koji NakanishiMaria Jasin
Feb 5, 2005·The EMBO Journal·Joanna M PrasherLaura J Niedernhofer
Aug 24, 2005·Nature Genetics·Marieke LevitusHans Joenje
Aug 24, 2005·Nature Genetics·Orna LevranArleen D Auerbach
Aug 24, 2005·Nature Structural & Molecular Biology·Georgina MosedaleKetan J Patel
Dec 27, 2005·Cell·Laura J NiedernhoferJan H J Hoeijmakers
Apr 29, 2006·British Journal of Haematology·Blanche P Alter
Jul 25, 2006·Molecular Therapy : the Journal of the American Society of Gene Therapy·Susana NavarroJuan A Bueren
Dec 7, 2006·The Journal of Biological Chemistry·Elda CannavoJosef Jiricny
Jan 4, 2007·Nature Genetics·Bing XiaJohan P de Winter
Sep 23, 2008·Cell·Markus RäschleJohannes C Walter

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Citations

Oct 5, 2019·Experimental Biology and Medicine·Muriel W Lambert
Mar 4, 2020·Genes·Robert M Brosh, Steven W Matson
Feb 26, 2020·Genes·Sara OvejeroMaría P Sacristán
Aug 14, 2020·Genes·Alice FinardiRosella Visintin
Jan 11, 2020·Scientific Reports·Marcello GermoglioAdele Adamo
Dec 22, 2020·The Journal of Cell Biology·Riko IshimotoMasatoshi Fujita
Jan 28, 2021·Cell Reports·Arik TownsendHuzefa Dungrawala
Feb 24, 2021·Molecular Cancer Research : MCR·Jennifer A CalvoSharon B Cantor
Sep 1, 2021·Cellular and Molecular Life Sciences : CMLS·Kazumasa Yoshida, Masatoshi Fujita
Aug 30, 2021·Molecular Cancer·Salvatore BenfattoBalca R Mardin
Jan 20, 2022·Human Reproduction Update·Valeria Stella VanniRaoul Orvieto

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Methods Mentioned

BETA
chromosomal aberrations
dissection
tandem affinity purification
ubiquitination
proximity ligation assay
immunoprecipitation

Software Mentioned

iPOND
FANCJ

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