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- Title
FANCE: the link between Fanconi anaemia complex assembly and activity.
- Authors
Pace, Paul; Johnson, Mark; Tan, Wu Meng; Mosedale, Georgina; Sng, Chelvin; Hoatlin, Maureen; de Winter, Johan; Joenje, Hans; Gergely, Fanni; Patel, K J
- 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.
- Publication
The EMBO journal, 2002, Vol 21, Issue 13, p3414
- ISSN
0261-4189
- Publication type
Journal Article
- DOI
10.1093/emboj/cdf355