Requirement of yeast RAD2, a homolog of human XPG gene, for efficient RNA polymerase II transcription. implications for Cockayne syndrome

Cell. 2002 Jun 28;109(7):823-34. doi: 10.1016/s0092-8674(02)00795-x.

Abstract

In addition to xeroderma pigmentosum, mutations in the human XPG gene cause early onset Cockayne syndrome (CS). Here, we provide evidence for the involvement of RAD2, the S. cerevisiae counterpart of XPG, in promoting efficient RNA polymerase II transcription. Inactivation of RAD26, the S. cerevisiae counterpart of the human CSB gene, also causes a deficiency in transcription, and a synergistic decline in transcription occurs in the absence of both the RAD2 and RAD26 genes. Growth is also retarded in the rad2 Delta and rad26 Delta single mutant strains, and a very severe growth inhibition is seen in the rad2 Delta rad26 Delta double mutant. From these and other observations presented here, we suggest that transcriptional defects are the underlying cause of CS.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Cell Cycle Proteins*
  • Cell Division
  • Cockayne Syndrome / metabolism*
  • DNA-Binding Proteins / chemistry*
  • Endodeoxyribonucleases*
  • Endonucleases
  • Fungal Proteins / chemistry
  • Fungal Proteins / genetics
  • Fungal Proteins / metabolism*
  • Galactose / metabolism
  • Gene Expression Regulation, Fungal
  • Genes, Fungal / genetics
  • Humans
  • Mutation
  • Nuclear Proteins
  • RNA Polymerase II / metabolism*
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae / genetics*
  • Saccharomyces cerevisiae / growth & development
  • Saccharomyces cerevisiae Proteins / chemistry
  • Saccharomyces cerevisiae Proteins / genetics
  • Saccharomyces cerevisiae Proteins / metabolism
  • Schizosaccharomyces pombe Proteins*
  • Time Factors
  • Transcription Factors / genetics
  • Transcription Factors / metabolism
  • Transcription Factors, General*
  • Transcription, Genetic*
  • Transcriptional Elongation Factors*

Substances

  • Cell Cycle Proteins
  • DNA excision repair protein ERCC-5
  • DNA-Binding Proteins
  • Fungal Proteins
  • Nuclear Proteins
  • RNA, Messenger
  • Saccharomyces cerevisiae Proteins
  • Schizosaccharomyces pombe Proteins
  • Transcription Factors
  • Transcription Factors, General
  • Transcriptional Elongation Factors
  • rad26 protein, S pombe
  • transcription factor S-II
  • RAD2 protein, S cerevisiae
  • RNA Polymerase II
  • Endodeoxyribonucleases
  • Endonucleases
  • Galactose