Defective interplay of activators and repressors with TFIIH in xeroderma pigmentosum

Juhong Liu, Sasha Akoulitchev, Achim Weber, Hui Ge, Sergei Chuikov, Daniel Libutti, Xin W. Wang, Joan Weliky Conaway, Curtis C. Harris, Ronald C. Conaway, Danny Reinberg, David Levens

Research output: Contribution to journalArticlepeer-review

110 Scopus citations


Inherited mutations of the TFIIH helicase subunits xeroderma pigmentosum (XP) B or XPD yield overlapping DNA repair and transcription syndromes. The high risk of cancer in these patients is not fully explained by the repair defect. The transcription defect is subtle and has proven more difficult to evaluate. Here, XPB and XPD mutations are shown to block transcription activation by the FUSE Binding Protein (FBP), a regulator of c-myc expression, and repression by the FBP Interacting Repressor (FIR). Through TFIIH, FBP facilitates transcription until promoter escape, whereas after initiation, FIR uses TFIIH to delay promoter escape. Mutations in TFIIH that impair regulation by FBP and FIR affect proper regulation of c-myc expression and have implications in the development of malignancy.

Original languageEnglish (US)
Pages (from-to)353-363
Number of pages11
Issue number3
StatePublished - Feb 9 2001
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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