Reduced RNA polymerase II transcription in extracts of cockayne syndrome and xeroderma pigmentosum/Cockayne syndrome cells.

AUTOR(ES)

Dianov, G L

RESUMO

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.

Documentos Relacionados

• Xeroderma pigmentosum--Cockayne syndrome complex: a further case.
• Reduced RNA polymerase II transcription in intact and permeabilized Cockayne syndrome group B cells
• Oxidative damage-induced PCNA complex formation is efficient in xeroderma pigmentosum group A but reduced in Cockayne syndrome group B cells.
• UV-induced ubiquitination of RNA polymerase II: a novel modification deficient in Cockayne syndrome cells.
• Defective thymine dimer excision by cell-free extracts of xeroderma pigmentosum cells.