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The high response rate to treatment with IBAT (odevixibat) in these patients suggests that in PFIC4, despite tight junction dysfunction, there is a regular passage of BAs from the hepatocytes to the biliary tree and the bowel, allowing odevixibat to interrupt the enterohepatic circulation and decrease the recirculating BA pool [1, 42, 47]

C: RT-qPCR analysis of Mitf, Tyr, Dct, Fosl1, and Mmp3 in B16F1 and D4M3A.781 wildtype or Gclc knockout cells

Fast for six hours before abdominal treatments

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