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Prepublished online as a Blood First Edition Paper on June 7, 2002; DOI 10.1182/blood-2002-03-0860.
Submitted April 8, 2002
Accepted May 20, 2002
The Fanconi anemia protein, FANCE, promotes the nuclear accumulation of FANCC
Toshiyasu Taniguchi and Alan D D'Andrea*
Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA; Department of Pediatrics, Children's Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA
* Corresponding author; email: Alan_Dandrea{at}dfci.harvard..
Fanconi Anemia is an autosomal recessive disorder characterized by aplastic anemia, cancer susceptibility, and cellular sensitivity to mitomycin C. The six known Fanconi Anemia gene products (FANCA, FANCC, FANCD2, FANCE, FANCF and FANCG proteins) interact in a common pathway. The monoubiquitination and nuclear foci formation of FANCD2 are essential for the function of this pathway. FANCA, FANCC, FANCG and FANCF proteins form a multisubunit nuclear complex (FA complex) required for FANCD2 monoubiquitination. Since FANCE and FANCC interact in vitro and FANCE is required for the FANCD2 monoubiquitination, we reasoned that FANCE is a component of the FA complex in vivo. Here we demonstrate that retroviral transduction of FA-E cells with the FANCE cDNA restores the nuclear accumulation of FANCC protein, FANCA-FANCC complex formation, the monoubiquitination and nuclear foci formation of FANCD2, and mitomycin C resistance. HA-tagged FANCE protein localizes diffusely in the nucleus. In normal cells, HA-tagged FANCE protein co-immunoprecipitates with FANCA, FANCC, and FANCG but not with FANCD2. Our data indicate that FANCE is a component of the nuclear FA complex in vivo and is required for the monoubiquitination of FANCD2 and the downstream events in the FA pathway.
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