Reduced RNA polymerase II transcription in extracts of Cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells

Grigory L. Dianov, Jean François Houle, Narayan Iyer, Vilhelm A. Bohr, Errol C. Friedberg

Research output: Contribution to journalArticle

59 Citations (Scopus)

Abstract

The hereditary disease Cockayne syndrome (CS) is a complex clinical syndrome characterized by arrested post-natal growth as well as neurological and other defects. The CSA and CSB genes are implicated in this disease. The clinical features of CS can also accompany the excision repair-defective hereditary disorder xeroderma pigmentosum (XP) from genetic complementation groups B, D or G. The XPB and XPD proteins are subunits of RNA polymerase II (RNAP II) transcription factor IIH (TFIIH). We show here that extracts of CS-A and CS-B cells, as well as those from XP-B/CS cells, support reduced levels of RNAP II transcription in vitro and that this feature is dependent on the state or quality of the template.

Original languageEnglish (US)
Pages (from-to)3636-3642
Number of pages7
JournalNucleic Acids Research
Volume25
Issue number18
DOIs
StatePublished - Sep 15 1997

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Cockayne Syndrome
Xeroderma Pigmentosum
RNA Polymerase II
Transcription Factor TFIIH
Inborn Genetic Diseases
Protein Subunits
DNA Repair
B-Lymphocytes
Growth
Genes

ASJC Scopus subject areas

  • Genetics

Cite this

Reduced RNA polymerase II transcription in extracts of Cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells. / Dianov, Grigory L.; Houle, Jean François; Iyer, Narayan; Bohr, Vilhelm A.; Friedberg, Errol C.

In: Nucleic Acids Research, Vol. 25, No. 18, 15.09.1997, p. 3636-3642.

Research output: Contribution to journalArticle

Dianov, Grigory L. ; Houle, Jean François ; Iyer, Narayan ; Bohr, Vilhelm A. ; Friedberg, Errol C. / Reduced RNA polymerase II transcription in extracts of Cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells. In: Nucleic Acids Research. 1997 ; Vol. 25, No. 18. pp. 3636-3642.
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