title: The Fanconi anemia pathway in replication stress and DNA crosslink repair.
abstract: Interstand crosslinks (ICLs) are DNA lesions where the bases of opposing DNA strands are covalently linked, inhibiting critical cellular processes such as transcription and replication. Chemical agents that generate ICLs cause chromosomal abnormalities including breaks, deletions and rearrangements, making them highly genotoxic compounds. This toxicity has proven useful for chemotherapeutic treatment against a wide variety of cancer types. The majority of our understanding of ICL repair in humans has been uncovered through analysis of the rare genetic disorder Fanconi anemia, in which patients are extremely sensitive to crosslinking agents. Here, we discuss recent insights into ICL repair gained using new repair assays and highlight the role of the Fanconi anemia repair pathway during replication stress.
authors: Department of Biochemistry, New York University School of Medicine, 550 First Ave., MSB 399, New York, NY, 10016, USA.
authors: Mathew J K
authors: MJ
authors: Jones
authors: Tony T
authors: TT
authors: Huang
mesh_terms: D003432:Cross-Linking Reagents / Q000737:chemistry; D004247:DNA / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D004249:DNA Damage; D004260:DNA Repair / Q000235:genetics*; D004261:DNA Replication / Q000235:genetics*; D005199:Fanconi Anemia / Q000235:genetics* / Q000378:metabolism; D051856:Fanconi Anemia Complementation Group Proteins / Q000378:metabolism; D006801:Humans; D008957:Models, Genetic; D015398:Signal Transduction / Q000235:genetics*
chemical_list: D003432:Cross-Linking Reagents; D051856:Fanconi Anemia Complementation Group Proteins; D004247:DNA