Mutations in any of at least sixteen FANC genes (FANCA-Q) cause Fanconi anemia, 
a disorder characterized by sensitivity to DNA interstrand crosslinking agents. 
The clinical features of cytopenia, developmental defects, and tumor 
predisposition are similar in each group, suggesting that the gene products 
participate in a common pathway. The Fanconi anemia DNA repair pathway consists 
of an anchor complex that recognizes damage caused by interstrand crosslinks, a 
multisubunit ubiquitin ligase that monoubiquitinates two substrates, and several 
downstream repair proteins including nucleases and homologous recombination 
enzymes. We review progress in the use of structural and biochemical approaches 
to understanding how each FANC protein functions in this pathway.