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

doi:10.1016/S0092-8674(01)00223-9

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 journal › Article › peer-review

111 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	353-363
Number of pages	11
Journal	Cell
Volume	104
Issue number	3
DOIs	https://doi.org/10.1016/S0092-8674(01)00223-9
State	Published - Feb 9 2001
Externally published	Yes

ASJC Scopus subject areas

General Biochemistry, Genetics and Molecular Biology

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10.1016/S0092-8674(01)00223-9

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title = "Defective interplay of activators and repressors with TFIIH in xeroderma pigmentosum",

abstract = "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.",

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

note = "Funding Information: We thank Dr. Kenneth Kraemer for providing XPD (XP6BE) cells and wild-type XPD and XPB expression plasmids. We thank Thomas Ried for use of his microscope. These studies were supported in part by grants to D. R. (NIH GM37120 and GM48518) and R. C. C. (R37 GM41628). J. W. C. is an Associate Investigator of the Howard Hughes Medical Institute. D. R. is an Investigator of the Howard Hughes Medical Institute. A. W. was supported in part by a grant from the Deutsche Forschungsgemeinschaft (We 2397/1-1).",

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doi = "10.1016/S0092-8674(01)00223-9",

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T1 - Defective interplay of activators and repressors with TFIIH in xeroderma pigmentosum

AU - Liu, Juhong

AU - Akoulitchev, Sasha

AU - Weber, Achim

AU - Ge, Hui

AU - Chuikov, Sergei

AU - Libutti, Daniel

AU - Wang, Xin W.

AU - Conaway, Joan Weliky

AU - Harris, Curtis C.

AU - Conaway, Ronald C.

AU - Reinberg, Danny

AU - Levens, David

N1 - Funding Information: We thank Dr. Kenneth Kraemer for providing XPD (XP6BE) cells and wild-type XPD and XPB expression plasmids. We thank Thomas Ried for use of his microscope. These studies were supported in part by grants to D. R. (NIH GM37120 and GM48518) and R. C. C. (R37 GM41628). J. W. C. is an Associate Investigator of the Howard Hughes Medical Institute. D. R. is an Investigator of the Howard Hughes Medical Institute. A. W. was supported in part by a grant from the Deutsche Forschungsgemeinschaft (We 2397/1-1).

PY - 2001/2/9

Y1 - 2001/2/9

N2 - 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.

AB - 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.

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Defective interplay of activators and repressors with TFIIH in xeroderma pigmentosum

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