基因名：
ERCC8
产品别名：
CKN1； CSA； UVSS2； ERCC8； ERCC excision repair 8, CSA ubiquitin ligase complex subunit； ERCC excision repair 8, CSA ubiquitin ligase complex subunit； DNA excision repair protein ERCC-8； Cockayne syndrome WD-repeat protein CSA； cockayne syndrome WD repeat protein CSA； excision repair cross-complementation group 8； excision repair cross-complementing rodent repair deficiency, complementation group 8； 剪切修复交叉互补修复缺陷偶联因子8(ERCC8)； 科凯恩氏综合症相关蛋白/早衰蛋白CSA；
背景信息：
Nucleotide excision repair of DNA lesions occurs more rapidly and at a higher frequency on the template, or the transcribed, strand of DNA and to a much lesser extent on the coding, or the non-transcribed, strand or on transcriptionally inactive DNA. CSA and CSB are two related genes that are responsible for directing this preferential DNA repair pattern, known as transcriptional-repair coupling. Cells from patients with the UV-sensitive nucleotide excision repair disorder Cockayne's syndrome (CS) have specific mutations affecting these genes and results in defects of the preferential repair on the transcribed strand of activated genes. CSA is a protein that belongs in the "WD-repeat" family of proteins. CSB, which is also designated excision repair cross-complementing protein-6 (ERCC-6), is the homolog of the yeast Rad26 protein. CSB belongs in the SWI/SNF family of proteins as it contains helicase motifs and ATPase activity.
