The DOI matches its Crossref record; 70 of the 71 references it cites were found in a registry or an index.
Interaction of the Fanconi Anemia Proteins and BRCA1 in a Common Pathway
Open accessAbstract
Fanconi anemia (FA) is a human autosomal recessive cancer susceptibility disorder characterized by cellular sensitivity to mitomycin C and ionizing radiation. Although six FA genes (for subtypes A, C, D2, E, F, and G) have been cloned, their relationship to DNA repair remains unknown. In the current study, we show that a nuclear complex containing the FANCA, FANCC, FANCF, and FANCG proteins is required for the activation of the FANCD2 protein to a monoubiquitinated isoform. In normal (non-FA) cells, FANCD2 is monoubiquitinated in response to DNA damage and is targeted to nuclear foci (dots). Activated FANCD2 protein colocalizes with the breast cancer susceptibility protein, BRCA1, in ionizing radiation-induced foci and in synaptonemal complexes of meiotic chromosomes. The FANCD2 protein, therefore, provides the missing link between the FA protein complex and the cellular BRCA1 repair machinery. Disruption of this pathway results in the cellular and clinical phenotype common to all FA subtypes.
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“Since BRCA1 interacts with FancD2 and mediates FancD2 foci formation after treatment with MMC (Bunting et al., 2012; Garcia-Higuera et al., 2001; Vandenberg et al., 2003)”
From The BRCA1-Interacting Protein Abraxas Is Required for Genomic Stability and Tumor Suppression (2014) · cites this paper as Garcia-Higuera et al., 2001
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“In addition, DNA damage induces the accumulation of BRCA-FA proteins in nuclear foci [33].”
From Hereditary Breast Cancer and the BRCA1-Associated FANCJ/BACH1/BRIP1 (2011) · cites this paper as [33]
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Citations
Cites
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The Ubiquitin-Proteasome Proteolytic Pathway: Destruction for the Sake of Construction
Glickman, M. H., & Ciechanover, A. J. (2002) · Physiological Reviews · doi:10.1152/physrev.00027.2001 · Cited by 4,304
Cell cycle checkpoint signaling through the ATM and ATR kinases
Abraham, R. T. (2001) · Genes & Development · doi:10.1101/gad.914401 · Cited by 1,966
Cancer Susceptibility and the Functions of BRCA1 and BRCA2
Venkitaraman, A. R. (2002) · Cell · doi:10.1016/s0092-8674(02)00615-3 · Cited by 1,713
Hallmarks of 'BRCAness' in sporadic cancers
Turner, N. C., Tutt, A. N. J., & Ashworth, A. (2004) · Nature reviews. Cancer · doi:10.1038/nrc1457 · Cited by 1,690
Ubiquitin: structures, functions, mechanisms
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Metadata
- Authors
- Garcia-Higuera, I., Taniguchi, T., Ganesan, S., Meyn, M. S., Timmers, C., Hejna, J., Grompe, M., & D'Andrea, A. D.
- Affiliations
- Harvard University (US)
- Dana-Farber Cancer Institute (US)
- Hospital for Sick Children (CA)
- Oregon Health & Science University (US)
- Venue
- Molecular Cell
- Publisher
- Elsevier BV
- Published
- 2001-02
- Volume
- 7
- Issue
- 2
- Pages
- 249-262
- ISSN
- 1097-2765 (ISSN-L)
- OpenAlex
- W1991841649
- Cited by
- 1,198
- References
- 71
- Citations by year
- 2026: 14 · 2025: 13 · 2024: 14 · 2023: 20 · 2022: 15 · 2021: 24 · 2020: 25 · 2019: 41 · 2018: 28 · 2017: 20 · 2016: 54 · 2015: 49 · 2014: 51 · 2013: 44 · 2012: 66
- FWCI
- 31.72
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Molecular Cell (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Heart, Lung, and Blood Institute (P01HL54785-04, R01HL52725-04, R01HL052725)
- National Institute of Diabetes and Digestive and Kidney Diseases (R01DK043889, R01DK43889-09)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Health Sciences › Medicine › Hematology › Blood disorders and treatments
- Health Sciences › Medicine › Oncology › PARP inhibition in cancer therapy
- Keywords
- Fanconi anemia, BRCA1, FANCD2 monoubiquitination, FANCA, FANCC, FANCF, FANCG, DNA repair, radiosensitivity, mitomycin C sensitivity, synaptonemal complex, meiotic chromosomes, nuclear foci, cancer susceptibility
- MeSH terms
- Animals, Cell Line, Cell Survival, DNA Damage, DNA-Binding Proteins, Fanconi Anemia, Fluorescent Antibody Technique, Genetic Complementation Test, Humans, Male, Meiosis, Nuclear Proteins, Protein Binding, Proteins, Radiation, Ionizing, Spermatocytes, Synaptonemal Complex, Ubiquitins, Ultraviolet Rays, Mitomycin, Cell Cycle Proteins, BRCA1 Protein, Active Transport, Cell Nucleus, Macromolecular Substances, Mice, Fanconi Anemia Complementation Group Proteins, Fanconi Anemia Complementation Group C Protein
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