FANCE: the Link Between Fanconi Anaemia Complex Assembly and Activity

Overview

Journal EMBO J

Specialties Biotechnology
Molecular Biology

Date 2002 Jul 3

PMID 12093742

Citations 46

Authors

Paul Pace

Mark Johnson

Wu Meng Tan

Georgina Mosedale

Chelvin Sng

Maureen Hoatlin

Johan de Winter

Hans Joenje

Fanni Gergely

K J Patel

Affiliations

Soon will be listed here.

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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