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Mutations in particular genes encoding these transportersincluding BSEP ( ABCB11 gene), MDR3 ( ABCB4 gene), sterolin-1 and sterolin-2 ( ABCG5/8 genes), and MRP2 ( ABCC2 gene)result in a wide spectrum of liver diseases, ranging from benign conditions such as Dubin-Johnson syndrome to more severe presentations like progressive familial intrahepatic cholestasis