In addition to gluconeogenesis, liver-specific LKB-defective mice present with severe cholestasis due to a lack of BSEP membrane-localization in the liver [54], while the apical side of the dilated canalicular structure was positive for BSEP in the livers of Sik3−/− mice, suggesting that the cause of cholestasis in these mice may not be identical to that in LKB-defective mice. The gene discussed is SIK3; the disease is cholestasis.