Proteins encoded by five of the six known Fanconi anemia (FA) genes form a heteromeric
complex that facilitates repair of DNA damage induced by cross-linking agents. A certain
number of these proteins, notably FANCC, also function independently to modulate
apoptotic signaling, at least in part, by suppressing ground state activation of the pro-
apoptotic interferon-inducible double-stranded RNA-dependent protein kinase (PKR).
Because certain FANCC mutations interdict its anti-apoptotic function without interfering with …