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Learn MoreCockayne syndrome (CS) is mainly caused by mutations in CSB gene encoding a protein belonging to SWI/SNF chromatin remodeling family. CS1AN cells derived from CS patients carrying mutations in CSB gene are useful for the study of nucleotide excision repair (NER) upon UV- or oxidative stress-induced DNA damage. However, establishment of isogenic cells endogenously expressing wild type CSB is tedious and difficult. Normal fibroblast MRC5 has been widely used to study cellular reponse to stress. This study was designed to systematically analyze the features of these two cell lines during DNA damage and repair pathways, aiming to provide a reference for application of these two cell lines as in vitro model. We show that CS1AN is hypersensitive to UV irradiation compared to MRC5. We found that CSB is essential for regulating gene expression in response to DNA damage. SOURCE: Yuming Wang (wangyuming2006@hotmail.com) - Guangzhou medical university
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