ArticleBlood2012
Regulation of the Fanconi anemia pathway by a CUE ubiquitin-binding domain in the FANCD2 protein.
Article in Blood, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 34 citations in OpenAlex.
- The role of CUEDC1 in suppressing JAK1/STAT3 signaling pathway in esophageal cancer.Scientific reports · 2026Article
- Expression and clinical significance of FANCI gene in pan-cancer: a comprehensive analysis based on multi-omics data.Frontiers in genetics · 2025Article
- The role of ubiquitination in health and disease.MedComm · 2024Review
- FANCD2-dependent mitotic DNA synthesis relies on PCNA K164 ubiquitination.Cell reports · 2023Article
- Fanconi anemia: current insights regarding epidemiology, cancer, and DNA repair.Human genetics · 2022Review
- Chromatin Ubiquitination Guides DNA Double Strand Break Signaling and Repair.Frontiers in cell and developmental biology · 2022Review
- Differential functions of FANCI and FANCD2 ubiquitination stabilize ID2 complex on DNA.EMBO reports · 2020Article
- A distinct role for recombination repair factors in an early cellular response to transcription-replication conflicts.Nucleic acids research · 2020Article
- Article
- FANCD2 Binding to H4K20me2 via a Methyl-Binding Domain Is Essential for Efficient DNA Cross-Link Repair.Molecular and cellular biology · 2019Article
- Fanconi anemia protein FANCI functions in ribosome biogenesis.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Understanding the Histone DNA Repair Code: H4K20me2 Makes Its Mark.Molecular cancer research : MCR · 2018Review
- Article
- The Simple Chordate Ciona intestinalis Has a Reduced Complement of Genes Associated with Fanconi Anemia.Evolutionary bioinformatics online · 2016Article
- ATR-mediated phosphorylation of FANCI regulates dormant origin firing in response to replication stress.Molecular cell · 2015Article
- p53 mediated apoptosis in osteosarcoma MG-63 cells by inhibition of FANCD2 gene expression.International journal of clinical and experimental medicine · 2015Article
- The Fanconi anemia ID2 complex: dueling saxes at the crossroads.Cell cycle (Georgetown, Tex.) · 2014Review
- Coordinate nuclear targeting of the FANCD2 and FANCI proteins via a FANCD2 nuclear localization signal.PloS one · 2013Article
- Ubiquitin and SUMO pathways in DNA replication and replication-coupled repair.Critical reviews in biochemistry and molecular biologyReview
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
The Fanconi anemia (FA)-BRCA pathway is critical for the repair of DNA interstrand crosslinks (ICLs) and the maintenance of chromosome stability. A key step in FA-BRCA pathway activation is the covalent attachment of monoubiquitin to FANCD2 and FANCI. Monoubiquitinated FANCD2 and FANCI localize in chromatin-associated nuclear foci where they interact with several well-characterized DNA repair proteins. Importantly, very little is known about the structure, function, and regulation of FANCD2. Herein, we describe the identification and characterization of a CUE (coupling of ubiquitin conjugation to endoplasmic reticulum degradation) ubiquitin-binding domain (UBD) in FANCD2, and demonstrate that the CUE domain mediates noncovalent binding to ubiquitin in vitro. We show that although mutation of the CUE domain destabilizes FANCD2, the protein remains competent for DNA damage-inducible monoubiquitination and phosphorylation. Importantly, we demonstrate that the CUE domain is required for interaction with FANCI, retention of monoubiquitinated FANCD2, and FANCI in chromatin, and for efficient ICL repair. Our results suggest a model by which heterodimerization of monoubiquitinated FANCD2 and FANCI in chromatin is mediated in part through a noncovalent interaction between the FANCD2 CUE domain and monoubiquitin covalently attached to FANCI, and that this interaction shields monoubiquitinated FANCD2 from polyubiquitination and proteasomal degradation.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.