Abstract
The hereditary disease Cockayne syndrome (CS) is characterized by a complex clinical phenotype. CS cells are abnormally sensitive to ultraviolet radiation and are defective in the repair of transcriptionally active genes. The cloned CSB gene encodes a member of a protein family that includes the yeast Snf2 protein, a component of the transcriptional 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 factor IIH. These observations suggest that the products of the CSA and CSB genes are involved in transcription.
Original language | English (US) |
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Pages (from-to) | 555-564 |
Number of pages | 10 |
Journal | Cell |
Volume | 82 |
Issue number | 4 |
DOIs | |
State | Published - Aug 25 1995 |
ASJC Scopus subject areas
- General Biochemistry, Genetics and Molecular Biology