Fance: The link between Fanconi anaemia complex assembly and activity

Paul Pace; Mark Johnson; Wu Meng Tan; Georgina Mosedale; Chelvin Sng; Maureen Hoatlin; Johan De Winter; Hans Joenje; Fanni Gergely; K. J. Patel

doi:10.1093/emboj/cdf355

Fance: The link between Fanconi anaemia complex assembly and activity

Paul Pace, Mark Johnson, Wu Meng Tan, Georgina Mosedale, Chelvin Sng, Maureen Hoatlin, Johan De Winter, Hans Joenje, Fanni Gergely, K. J. Patel

Research output: Contribution to journal › Article › peer-review

139 Scopus citations

Abstract

The Fanconi anaemia (FA) nuclear complex (composed of the FA proteins A, C, G and F) is essential for protection against chromosome breakage. It activates the downstream protein FANCD2 by monoubiquitylation; this then forges an association with the BRCA1 protein at sites of DNA damage. Here we show that the recently identified FANCE protein is part of this nuclear complex, binding both FANCC and FANCD2. Indeed, FANCE is required for the nuclear accumulation of FANCC and provides a critical bridge between the FA complex and FANCD2. Disease-associated FANCC mutants do not bind to FANCE, cannot accumulate in the nucleus and are unable to prevent chromosome breakage.

Original language	English (US)
Pages (from-to)	3414-3423
Number of pages	10
Journal	EMBO Journal
Volume	21
Issue number	13
DOIs	https://doi.org/10.1093/emboj/cdf355
State	Published - Jul 1 2002
Externally published	Yes

Keywords

Chromosome breakage
FANCE
Fanconi anaemia
Nuclear complex assembly

ASJC Scopus subject areas

General Neuroscience
Molecular Biology
General Biochemistry, Genetics and Molecular Biology
General Immunology and Microbiology

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10.1093/emboj/cdf355

Cite this

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title = "Fance: The link between Fanconi anaemia complex assembly and activity",

abstract = "The Fanconi anaemia (FA) nuclear complex (composed of the FA proteins A, C, G and F) is essential for protection against chromosome breakage. It activates the downstream protein FANCD2 by monoubiquitylation; this then forges an association with the BRCA1 protein at sites of DNA damage. Here we show that the recently identified FANCE protein is part of this nuclear complex, binding both FANCC and FANCD2. Indeed, FANCE is required for the nuclear accumulation of FANCC and provides a critical bridge between the FA complex and FANCD2. Disease-associated FANCC mutants do not bind to FANCE, cannot accumulate in the nucleus and are unable to prevent chromosome breakage.",

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AU - Pace, Paul

AU - Johnson, Mark

AU - Tan, Wu Meng

AU - Mosedale, Georgina

AU - Sng, Chelvin

AU - Hoatlin, Maureen

AU - De Winter, Johan

AU - Joenje, Hans

AU - Gergely, Fanni

AU - Patel, K. J.

PY - 2002/7/1

Y1 - 2002/7/1

N2 - The Fanconi anaemia (FA) nuclear complex (composed of the FA proteins A, C, G and F) is essential for protection against chromosome breakage. It activates the downstream protein FANCD2 by monoubiquitylation; this then forges an association with the BRCA1 protein at sites of DNA damage. Here we show that the recently identified FANCE protein is part of this nuclear complex, binding both FANCC and FANCD2. Indeed, FANCE is required for the nuclear accumulation of FANCC and provides a critical bridge between the FA complex and FANCD2. Disease-associated FANCC mutants do not bind to FANCE, cannot accumulate in the nucleus and are unable to prevent chromosome breakage.

AB - The Fanconi anaemia (FA) nuclear complex (composed of the FA proteins A, C, G and F) is essential for protection against chromosome breakage. It activates the downstream protein FANCD2 by monoubiquitylation; this then forges an association with the BRCA1 protein at sites of DNA damage. Here we show that the recently identified FANCE protein is part of this nuclear complex, binding both FANCC and FANCD2. Indeed, FANCE is required for the nuclear accumulation of FANCC and provides a critical bridge between the FA complex and FANCD2. Disease-associated FANCC mutants do not bind to FANCE, cannot accumulate in the nucleus and are unable to prevent chromosome breakage.

KW - Chromosome breakage

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KW - Fanconi anaemia

KW - Nuclear complex assembly

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Fance: The link between Fanconi anaemia complex assembly and activity

Abstract

Keywords

ASJC Scopus subject areas

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