Characterization of an induced pluripotent stem cell line (IMBPASi001-A) derived from fibroblasts of a patient affected by Wolfram Syndrome


Wolfram Syndrome is a rare, autosomal recessive genetic disorder with clinical symptoms appearing in early childhood. Here, we report a generation of iPSCs from fibroblasts of a patient affected by this disease. Induced pluripotent cells obtained with the application of integration-free episomal vectors display a normal human karyotype, express pluripotency markers, and are capable of differentiating into cells of the three embryonic germ layers. Thanks to these features, this cell line is a useful model for tissue-specific pathogenetic mechanisms in Wolfram Syndrome caused by WFS1 mutations. Copyright © 2020 The Author(s). Published by Elsevier B.V. All rights reserved.

Authors Grzela DP, Marciniak B, Pulaski L
Journal Stem cell research
Publication Date 2020 Jul;46:101858
PubMed 32521500
DOI 10.1016/j.scr.2020.101858

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