REDUCED RNA POLYMERASE II TRANSCRIPTION IN EXTRACTS OF COCKAYNE-SYNDROME AND XERODERMA PIGMENTOSUM/COCKAYNE-SYNDROME CELLS

摘要

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.