Mechanism of Ubiquitination and Deubiquitination in the Fanconi Anemia Pathway

Monoubiquitination and deubiquitination of FANCD2:FANCI heterodimer is central to DNA repair in a pathway that is defective in the cancer predisposition syndrome Fanconi anemia (FA). The “FA core complex” contains the RING-E3 ligase FANCL and seven other essential proteins that are mutated in variou...

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Bibliographic Details
Published in:	Molecular cell 2017-01, Vol.65 (2), p.247-259
Main Authors:	van Twest, Sylvie, Murphy, Vincent J., Hodson, Charlotte, Tan, Winnie, Swuec, Paolo, O’Rourke, Julienne J., Heierhorst, Jörg, Crismani, Wayne, Deans, Andrew J.
Format:	Article
Language:	English
Subjects:	Cell Line core complex deubiquitination DNA DNA - genetics DNA - metabolism DNA repair DNA-Binding Proteins - metabolism drug therapy enzyme mechanism FANCB FANCD2 Fanconi anemia Fanconi Anemia - genetics Fanconi Anemia - metabolism Fanconi Anemia Complementation Group A Protein - metabolism Fanconi Anemia Complementation Group C Protein - metabolism Fanconi Anemia Complementation Group D2 Protein - genetics Fanconi Anemia Complementation Group D2 Protein - metabolism Fanconi Anemia Complementation Group E Protein - metabolism Fanconi Anemia Complementation Group G Protein - metabolism Fanconi Anemia Complementation Group L Protein - metabolism Fanconi Anemia Complementation Group Proteins - genetics Fanconi Anemia Complementation Group Proteins - metabolism Fanconi syndrome Humans Inhibitor of Differentiation Protein 2 - metabolism monoubiquitination Multiprotein Complexes neoplasms Nuclear Proteins - metabolism Protein Binding Protein Multimerization receptors Recombinant Proteins - metabolism RING E3 Substrate Specificity Time Factors Transfection Ubiquitin-Specific Proteases - metabolism Ubiquitination
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