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Animal & Cellular Models and Pathophysiology

Etiology and pathogenesis of PSC are still unclear. Their improved understanding is necessary to devise novel treatment approaches and eventually cure the disease.

Efforts to reach this goal are hampered by several barriers:

1)     Lack of bona fide animal models of Primary Sclerosing Cholangitis (see Fickert’s paper on J. Hepatology for a discussion). There is however a number of models for Sclerosing Cholangitis that may generate useful information if properly used.

2)   Scarcity of human derived study material (specimens and cell lines). Isolation and culture of cholangiocytes form PSC livers, is possible, but made difficult by the low number of explants available form transplants and concerns about tissue sampling.

3)   Lower number of collaborative efforts among “translational” groups, as compared to the outstanding collaborative efforts of the “clinical” groups.

Novel technologies (such as iPSC-derived cholangiocytes, and biliary organoids) are likely to solve the problem of availability of human-derived and disease-specific cell lines.

The main aim of the Working Group is to improve collaboration and sharing of reagents among the translational labs.

An initiative for generation of human PSC cholangiocytes form iPSC and Organoid is in the work between Yale University, the University of Padova and the Medical University of Vienna (see enclosed slide presentation).