Cited by 823DNA Repair MechanismsBlood disorders and treatmentsBRCA gene mutations in cancer

The Fanconi anaemia/BRCA pathway

D'Andrea, Alan D., Grompe, Markus·2003·Nature Reviews Cancer

Abstract

Fanconi anaemia (FA) is a rare genetic cancer-susceptibility syndrome that is characterized by congenital abnormalities, bone-marrow failure and cellular sensitivity to DNA crosslinking agents. Seven FA-associated genes have recently been cloned, and their products were found to interact with well-known DNA-damage-response proteins, including BRCA1, ATM and NBS1. The FA proteins could therefore be involved in the cell-cycle checkpoint and DNA-repair pathways. Recent studies implicate the FA proteins in the process of repairing chromosome defects that occur during homologous recombination, and disruption of the FA genes results in chromosome instability--a common feature of many human cancers.

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Crossref · 10.1038/nrc970

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121 of 121 references verified0 citation errors · 0 not checkable

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  • Fanconi anemia patient cells are extremely sensitive to agents that induce DNA interstrand crosslinks.

    “FA is a rare cancer-prone disease in which patients’ cells are characterized by extreme sensitivity to agents that induce DNA interstrand crosslinks (for review, see [22,23]).”

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Authors
D'Andrea, A. D., & Grompe, M.
Affiliations
Oregon Health & Science University (US)
Venue
Nature Reviews Cancer
Published
2003-01
Volume
3
Issue
1
Pages
23-34
ISSN
  • 1474-175X (ISSN-L)
  • 1474-1768
OpenAlex
W2092950132
Cited by
822
References
122
Citations by year
2026: 3 · 2025: 14 · 2024: 18 · 2023: 15 · 2022: 12 · 2021: 21 · 2020: 15 · 2019: 16 · 2018: 23 · 2017: 26 · 2016: 43 · 2015: 30 · 2014: 37 · 2013: 26 · 2012: 32
FWCI
6.92
Type
journal article
Language
en
Open access
closed
Locations
  • Nature reviews. Cancer (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Blood disorders and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › BRCA gene mutations in cancer
Keywords
Fanconi anemia, BRCA1, DNA damage response, DNA repair, cell cycle checkpoints, ATM, NBS1, chromosomal instability, homologous recombination, bone marrow failure, cancer susceptibility genes
MeSH terms
Animals, Disease Models, Animal, DNA Repair, DNA-Binding Proteins, Fanconi Anemia, Genetic Complementation Test, Humans, Proteins, Signal Transduction, BRCA1 Protein, Chromosome Breakage, Mice, Fanconi Anemia Complementation Group A Protein

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