Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8)

ERCC8 encodes a WD repeat protein, which interacts with Cockayne syndrome type B (CSB) protein and with p44 protein, a subunit of the RNA polymerase II transcription factor IIH.Multiple alternatively spliced transcript variants encoding different isoforms have been found for this gene.In vitro translated CSA protein specifically interacted with the CSB (ERCC6) protein and p44 protein (GTF2H2), a subunit of the RNA polymerase II basal transcription factor TFIIH. These observations, coupled with the observation that the amino acid sequence of the predicted CSB polypeptide is homologous with a component of the yeast Swi/Snf transcriptional activation complex, suggested that Cockayne syndrome cells are defective in RNA polymerase II transcription.