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 language | English (US) |
---|---|
Pages (from-to) | 3636-3642 |
Number of pages | 7 |
Journal | Nucleic acids research |
Volume | 25 |
Issue number | 18 |
DOIs | |
State | Published - Sep 15 1997 |
ASJC Scopus subject areas
- Genetics