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doi: 10.1016/j.nbd.2025.106967 147 PosthumusTBEngelkeUFHAspersRMerxJBoltjeTJMartensJet al

Coverslips were then incubated with secondary (Alexa Flour 568 anti-mouse and Alexa Flour 488 anti-rabbit) 1:500 in 5% NDS for 1 h at room temperature in the dark and washed 3 times with PBS

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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]