title: The Fanconi anemia pathway and DNA interstrand cross-link repair.
abstract: Fanconi anemia (FA) is an autosomal or X-linked recessive disorder characterized by chromosomal instability, bone marrow failure, cancer susceptibility, and a profound sensitivity to agents that produce DNA interstrand cross-link (ICL). To date, 15 genes have been identified that, when mutated, result in FA or an FA-like syndrome. It is believed that cellular resistance to DNA interstrand cross-linking agents requires all 15 FA or FA-like proteins. Here, we review our current understanding of how these FA proteins participate in ICL repair and discuss the molecular mechanisms that regulate the FA pathway to maintain genome stability.
authors.affiliation: Life Sciences Institute, Zhejiang University, Hangzhou 310058, China.
authors.forename: Xiaoyu
authors.initials: X
authors.lastname: Su
authors.forename: Jun
authors.initials: J
authors.lastname: Huang
mesh_terms: D004249:DNA Damage; D004260:DNA Repair; D004706:Endodeoxyribonucleases; D005090:Exodeoxyribonucleases / Q000235:genetics / Q000378:metabolism; D005199:Fanconi Anemia / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D000076164:Fanconi Anemia Complementation Group N Protein; D051856:Fanconi Anemia Complementation Group Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D064251:Multifunctional Enzymes; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism; D045522:Recombinases / Q000235:genetics / Q000378:metabolism; D025521:Tumor Suppressor Proteins / Q000235:genetics / Q000378:metabolism; D054875:Ubiquitination
chemical_list: D000076164:Fanconi Anemia Complementation Group N Protein; D051856:Fanconi Anemia Complementation Group Proteins; D064251:Multifunctional Enzymes; D009687:Nuclear Proteins; C512790:PALB2 protein, human; D045522:Recombinases; D025521:Tumor Suppressor Proteins; D004706:Endodeoxyribonucleases; D005090:Exodeoxyribonucleases; C551063:FAN1 protein, human; C542319:SLX4 protein, human