Type 2 Progressive Familial Intrahepatic Cholestasis (PFIC-2), also known as BSEP disease, is one of the genetically determined cholestatic diseases and results from mutations in ABCB11, which encodes the bile salt export pump (BSEP) protein responsible for the bulk of conjugated bile salt transport from hepatocytes into biliary canaliculi. This evidence concerns the gene ABCB11 and progressive familial intrahepatic cholestasis type 2.