title: Reduced RNA polymerase II transcription in extracts of cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells.
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.
authors.affiliation: Laboratory of Molecular Pathology, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75235, USA.
authors.forename: G L
authors.initials: GL
authors.lastname: Dianov
authors.forename: J F
authors.initials: JF
authors.lastname: Houle
authors.forename: N
authors.initials: N
authors.lastname: Iyer
authors.forename: V A
authors.initials: VA
authors.lastname: Bohr
authors.forename: E C
authors.initials: EC
authors.lastname: Friedberg
mesh_terms: D002460:Cell Line; D003057:Cockayne Syndrome / Q000235:genetics*; D006801:Humans; D012319:RNA Polymerase II / Q000235:genetics*; D014158:Transcription, Genetic; D014983:Xeroderma Pigmentosum / Q000235:genetics*
chemical_list: D012319:RNA Polymerase II