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 journalArticle

131 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 languageEnglish (US)
Pages (from-to)3414-3423
Number of pages10
JournalEMBO Journal
Volume21
Issue number13
DOIs
StatePublished - Jul 1 2002

Keywords

  • Chromosome breakage
  • FANCE
  • Fanconi anaemia
  • Nuclear complex assembly

ASJC Scopus subject areas

  • Neuroscience(all)
  • Molecular Biology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Microbiology(all)

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    Pace, P., Johnson, M., Tan, W. M., Mosedale, G., Sng, C., Hoatlin, M., De Winter, J., Joenje, H., Gergely, F., & Patel, K. J. (2002). Fance: The link between Fanconi anaemia complex assembly and activity. EMBO Journal, 21(13), 3414-3423. https://doi.org/10.1093/emboj/cdf355