THE COCKAYNE-SYNDROME GROUP-A GENE ENCODES A WD REPEAT PROTEIN THAT INTERACTS WITH CSB PROTEIN AND A SUBUNIT OF RNA-POLYMERASE-II TFIIH

The hereditary disease Cockayne syndrome (CS) is characterized by a co mplex clinical phenotype, CS cells are abnormally sensitive to ultravi olet radiation and are defective in the repair of transcriptionally ac tive genes, The cloned CSB gene encodes a member of a protein family t hat includes the yeast Snf2 protein, a component of the transcriptiona l regulator Swi/Snf, We report the cloning of the CSA cDNA, which can encode a WD repeat protein, Mutations in the cDNA have been identified in CS-A cell lines, CSA protein. interacts with CSB protein and with p44 protein, a subunit of the human RNA polymerase II transcription fa ctor IIH. These observations suggest that the products of the CSA and CSB genes are involved in transcription.