,The Fanconi anemia pathway and DNA interstrand cross-link repair

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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.
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