The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH

Karla A. Henning, Lei Li, Narayan Iyer, Lisa D. McDaniel, Michael S. Reagan, Randy Legerski, Roger A. Schultz, Miria Stefanini, Alan R. Lehmann, Lynne V. Mayne, Errol C. Friedberg

Research output: Contribution to journalArticle

365 Scopus citations

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 languageEnglish (US)
Pages (from-to)555-564
Number of pages10
JournalCell
Volume82
Issue number4
DOIs
StatePublished - Aug 25 1995

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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    Henning, K. A., Li, L., Iyer, N., McDaniel, L. D., Reagan, M. S., Legerski, R., Schultz, R. A., Stefanini, M., Lehmann, A. R., Mayne, L. V., & Friedberg, E. C. (1995). The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH. Cell, 82(4), 555-564. https://doi.org/10.1016/0092-8674(95)90028-4