Collection of liver samples and clinical data from patients or donors was approved by the Swedish Ethical Review Authority (2017/269-31, 2017/1394–31). Samples from patients with severe Alagille syndrome (four) were obtained at time of liver transplant from extirpated liver. Samples were obtained with a consent to be used for research according to ethical permit 2017/269–31. Samples were dissociated for primary cell culture e.g. organoids (data not shown), and a matching sample was formalin fixed or fresh-frozen for comparative analyses. Liver tissue samples from patients with mild Alagille syndrome (six) were obtained for clinical follow-up purposes and were retrospectively analyzed. The liver material was obtained within the framework of clinical patient care can be analyzed retrospectively without the need for consent according to ethical permit 2017/1394–31. Healthy controls (two) were left-over donor material, or from organ donation post-mortem. The liver function tests were obtained during routine biochemical analyses.

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