Generation of an iPSC line (DPNJMUi002-A) from a progressive familial intrahepatic cholestasis 3 (PFIC3) patient with a heterozygous mutation in the ABCB4 gene

Summary

progressive familial intrahepatic cholestasis 3 (PFIC3) caused by mutations in ABCB4 gene is a rare lethal autosomal recessive liver disease. Here, we detail the reprogramming of peripheral blood mononuclear cells (PBMCs) using non-integrative Sendai virus to produce induced pluripotent stem cells (iPSCs) from a child carrying the ABCB4 mutation. The obtained iPSC presented normal karyotype, high expression of pluripotency markers and the capacity to differentiate into cells of three germ layers. Copyright © 2025 The Author(s). Published by Elsevier B.V. All rights reserved.

Authors Wu K, Wang C, Hu H, Shen Y, Zheng Y, Hu G, Liu Z
Journal Stem cell research
Publication Date 2025 Jun 5;87:103745
PubMed 40505422
DOI 10.1016/j.scr.2025.103745

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