The ubiquitination machinery of the Fanconi Anemia DNA repair pathway. (August 2021)

• Record Type: Journal Article
• Authors: Tan, Winnie; Deans, Andrew J.
• Is Part Of: Progress in biophysics and molecular biology. Volume 163(2021)
• Page Start: 5
• Page End: 13
• Publication Date: 2021-08
• Subjects: Ubiquitination -- Fanconi anemia -- DNA repair -- FA core complex -- FANCI-FANCD2 -- Interstrand crosslink repair; Biophysics -- Periodicals; Biochemistry -- Periodicals; Biophysics -- Periodicals; Molecular Biology -- Periodicals; Biophysique -- Périodiques; Biochimie -- Périodiques; 571.4
• DOI: 10.1016/j.pbiomolbio.2020.09.009
• Languages: English
• ISSNs: 0079-6107

Abstract

Abstract: The Fanconi Anemia (FA) pathway maintains genome stability by preventing DNA damage from occurring when replication is blocked. Central to the FA pathway is the monoubiquitination of FANCI-FANCD2 mediated by a ubiquitin RING-E3 ligase complex called the FA core complex. Genetic mutation in any component of the FA core complex results in defective FANCI-FANCD2 monoubiquitination and phenotypes of DNA damage sensitivity, birth defects, early-onset bone marrow failure and cancer. Here, we discuss the mechanisms of the FA core complex and FANCI-FANCD2 monoubiquitination at sites of blocked replication and review our current understanding of the biological functions of these proteins in replication fork protection.