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 sy ndrome characterized by arrested post-natal growth as well as neurolog ical and other defects, The CSA and CSB genes are implicated in this d isease, The clinical features of CS can also accompany the excision re pair-defective hereditary disorder xeroderma pigmentosum (XP) from gen etic complementation groups B, D or G. The XPB and XPD proteins are su bunits 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 t he template.