Generation of integration-free induced pluripotent stem cell (iPSC) line MURAi002-A from hemoglobin E/β-thalassemia disease patient harboring βE/β0 (CD41/42, –CTTT) compound heterozygous mutation

The HBB gene encodes the β-globin protein, a component of adult hemoglobin A (HbA) which is responsible for the transportation of oxygen. Mutations in the HBB gene can impair β-globin synthesis and disrupt hemoglobin production. Patients who possess both a protein-reducing β-thalassemia mutation and...

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Main Authors: Gunn Pornratananont, Amornrat Tangprasittipap, Chokdee Wongborisuth, Sukanya Chumchuen, Kanit Bhukhai, Usanarat Anurathapan, Suradej Hongeng, Duantida Songdej
Format: Article
Language:English
Published: Elsevier 2025-08-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S1873506125000935
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Summary:The HBB gene encodes the β-globin protein, a component of adult hemoglobin A (HbA) which is responsible for the transportation of oxygen. Mutations in the HBB gene can impair β-globin synthesis and disrupt hemoglobin production. Patients who possess both a protein-reducing β-thalassemia mutation and a βE mutation in their HBB gene are affected by hemoglobin E/β-thalassemia disease. This study demonstrates the successful generation and characterization of the human pluripotent stem cell (hiPSC) line MURAi002-A derived from a patient with hemoglobin E/β0-thalassemia disease harboring the specific codon 41/42 (−CTTT) β0-thalassemia mutation through the utilization of non-integrative reprogramming episomes.
ISSN:1873-5061