Since pharmacological inhibition of the TGFβ pathway improves bone marrow dysfunction, we hypothesized that a mouse model with disruption of Fancd2 and Smad3, the canonical mediator of the TGFβ pathway, would overcome this FA hematopoietic defect and perhaps increase the survival of DKO newborn mice. The gene discussed is FANCD2; the disease is Friedreich ataxia.