FANCI is a second monoubiquitinated member of the Fanconi anemia pathway

Ashley E. Sims, Elizabeth Spiteri, Robert J. Sims, Adriana G. Arita, Francis P. Lach, Thomas Landers, Melanie Wurm, Marcel Freund, Kornelia Neveling, Helmut Hanenberg, Arleen D. Auerbach, Tony T. Huang

Research output: Contribution to journalArticle

206 Scopus citations


Activation of the Fanconi anemia (FA) DNA damage-response pathway results in the monoubiquitination of FANCD2, which is regulated by the nuclear FA core ubiquitin ligase complex. A FANCD2 protein sequence-based homology search facilitated the discovery of FANCI, a second monoubiquitinated component of the FA pathway. Biallelic mutations in the gene coding for this protein were found in cells from four FA patients, including an FA-I reference cell line.

Original languageEnglish (US)
Pages (from-to)564-567
Number of pages4
JournalNature Structural and Molecular Biology
Issue number6
StatePublished - Jun 1 2007


ASJC Scopus subject areas

  • Structural Biology
  • Molecular Biology

Cite this

Sims, A. E., Spiteri, E., Sims, R. J., Arita, A. G., Lach, F. P., Landers, T., Wurm, M., Freund, M., Neveling, K., Hanenberg, H., Auerbach, A. D., & Huang, T. T. (2007). FANCI is a second monoubiquitinated member of the Fanconi anemia pathway. Nature Structural and Molecular Biology, 14(6), 564-567.