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Mutations in XPB and XPD helicases found in xeroderma pigmentosum patients impair the transcription function of TFIIH by Frédéric Coin, Etienne Bergmann,

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Presentation on theme: "Mutations in XPB and XPD helicases found in xeroderma pigmentosum patients impair the transcription function of TFIIH by Frédéric Coin, Etienne Bergmann,"— Presentation transcript:

1 Mutations in XPB and XPD helicases found in xeroderma pigmentosum patients impair the transcription function of TFIIH by Frédéric Coin, Etienne Bergmann, Alexandre Tremeau ‐ Bravard, and Jean ‐ Marc Egly EMBO J. Volume 18(5):1357-1366 March 1, 1999 ©1999 by European Molecular Biology Organization

2 Immunopurification of TFIIH using Ab ‐ p44. Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

3 Transcription and DNA repair activities of TFIIH(ip). Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

4 Composition and enzymatic activities of TFIIH isolated from HD2 cells containing a mutation in the XPD gene. Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

5 Composition and enzymatic activities of TFIIHs from patients with spinal muscular atrophy type I or II. TFIIH complexes isolated from patients with type I or II spinal muscular atrophy [IIH ‐ p44c and IIH ‐ p44(c+t), respectively] were immunopurifed and analyze... Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

6 Mutations in the XPB subunit differentially impede TFIIH transcription activity. Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

7 Artificial DNA promoter opening abolishes the transcription inhibition due to TFIIH of XPB patient. Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization

8 XPB mutations prevent promoter opening. Frédéric Coin et al. EMBO J. 1999;18:1357-1366 ©1999 by European Molecular Biology Organization


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