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Reduced RNA polymerase II transcription in extracts of Cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells
Author(s) -
Grigory L. Dianov,
Jean-François Houle,
N. Iyer,
Vilhelm A. Bohr,
E C Friedberg
Publication year - 1997
Publication title -
nucleic acids research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 9.008
H-Index - 537
eISSN - 1362-4954
pISSN - 0305-1048
DOI - 10.1093/nar/25.18.3636
Subject(s) - xeroderma pigmentosum , cockayne syndrome , transcription factor ii h , biology , nucleotide excision repair , complementation , rna polymerase ii , transcription (linguistics) , genetics , microbiology and biotechnology , dna repair , gene , gene expression , phenotype , promoter , linguistics , philosophy
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.

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