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

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 hered...

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Bibliographic Details
Published in:Nucleic acids research 1997-09, Vol.25 (18), p.3636-3642
Main Authors: Dianov, Grigory L., Houle, Jean-François, Iyer, Narayan, Bohr, Vilhelm A., Friedberg, Errol C.
Format: Article
Language:English
Subjects:
Cell Line
Cockayne Syndrome - genetics
Humans
RNA Polymerase II - genetics
Transcription, Genetic
Xeroderma Pigmentosum - genetics
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Summary: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.
ISSN:0305-1048
1362-4962
DOI:10.1093/nar/25.18.3636