Cystic Fibrosis (CF) is a genetic disease caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene which encodes for a chloride ion channel regulating the balance of salt and water across secretory epithelia. Here we generated an iPSC line from a CF patient homozygous for the p.Asn1303Lys mutation, a Class II folding defect mutation. This iPSC line provides a useful resource for disease modeling and to investigate the pharmacological response to CFTR modulators in iPSC derived epithelia.

中文翻译：

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从携带p.Asn1303Lys（N1303K）突变的囊性纤维化患者中诱导多能干细胞系（MHHi018-A）的产生。

囊性纤维化（CF）是一种遗传性疾病，是由囊性纤维化跨膜电导调节器（CFTR）基因突变引起的，该基因编码一个氯离子通道，调节整个分泌上皮中盐和水的平衡。在这里，我们从CF患者纯合的p.Asn1303Lys突变（II类折叠缺陷突变）中生成了一条iPSC品系。该iPSC品系为疾病建模和研究iPSC衍生的上皮细胞对CFTR调节剂的药理反应提供了有用的资源。