Review Volume 1, Issue 1 pp 28—37

Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A

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Figure 3. A proposed model showing that DNA double-strand break repair activity is impaired in HGPS and RD cells. Unlike the replication fork collapse induced by genotoxins, laminopathy-induced replication fork collapse may be characterized with a possible loss of PCNA at replication forks. The subsequent possible binding of XPA to the "naked" replication forks with DNA double-strand breaks (DSBs) blocks the access of DSB repair proteins to the damage sites. RFs stands for replication factors.