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, 82 (4), 555-64

The Cockayne Syndrome Group A Gene Encodes a WD Repeat Protein That Interacts With CSB Protein and a Subunit of RNA Polymerase II TFIIH

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The Cockayne Syndrome Group A Gene Encodes a WD Repeat Protein That Interacts With CSB Protein and a Subunit of RNA Polymerase II TFIIH

K A Henning et al. Cell.

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.

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