title: How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway.
abstract: Fanconi anemia (FA) is a rare human genetic disorder characterized by developmental defects, bone marrow failure and cancer predisposition, primarily due to a deficiency in the repair of DNA interstrand crosslinks (ICLs). ICL repair through the FA DNA repair pathway is a complicated multi-step process, involving at least 19 FANC proteins and coordination of multiple DNA repair activities, including homologous recombination, nucleotide excision repair and translesion synthesis (TLS). SUMOylation is a critical regulator of several DNA repair pathways, however, the role of this modification in controlling the FA pathway is poorly understood. Here, we summarize recent advances in the fine-tuning of the FA pathway by small ubiquitin-like modifier (SUMO)-targeted ubiquitin ligases (STUbLs) and other SUMO-related interactions, and discuss the implications of these findings in the design of novel therapeutics for alleviating FA-associated condition, including cancer.
authors.affiliation: Department of Biochemistry and Molecular Pharmacology, New York University School of Medicine, New York NY, USA.
authors.forename: Kate E
authors.initials: KE
authors.lastname: Coleman
authors.affiliation: Department of Biochemistry and Molecular Pharmacology, New York University School of Medicine, New York NY, USA.
authors.forename: Tony T
authors.initials: TT
authors.lastname: Huang
keywords: DNA interstrand crosslinks (ICLs); Fanconi anemia (FA); SUMO proteases (SENPs); SUMO-interaction motifs (SIMs); SUMO-like domains (SLDs); SUMO-targeted ubiquitin ligases (STUbLs); SUMOylation; deubiquitinases (DUBs)