Consistent with reduced FOXA2 expression in the liver of patients with cholestasis (89, 90), FOXA2-deficient mice exhibited bile acid accumulation and reduced expression of bile acid binding enzyme (Slc27a5), accompanied by transport protein defects due to the fact that FOXA2 directly regulates mrp2 and Oatp2 and indirectly regulates mrp3 and mrp4 (91). The gene discussed is FOXA2; the disease is cholestasis.