Marfan syndrome (MFS) is a connective-tissue disorder caused mainly by heterozygous mutations in the FBN1 gene that encodes fibrillin-1. In this study, human induced pluripotent stem cell (iPSC) line ZZUSAHi003-A was generated from peripheral blood mononuclear cells (PBMCs) isolated from a female patient with MFS using non-integrative Sendai virus. The iPSC line carried the FBN1 gene mutation, showed the normal karyotype, expressed pluripotency markers and had the capacity to differentiate into three germ layers in vivo. This iPS line, ZZUSAHi003-A, could serve as a useful tool for studying pathogenic mechanisms of MFS.

中文翻译：

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对人诱导的多能干细胞系进行重编程，该人系来自具有FBN1基因c.2939G> A杂合突变的Marfan综合征患者

马凡综合症（MFS）是一种结缔组织疾病，主要由编码原纤维蛋白1的FBN1基因中的杂合突变引起。在这项研究中，人类诱导的多能干细胞（iPSC）系ZZUSAHi003-A是使用非整合型仙台病毒从女性MFS患者的外周血单个核细胞（PBMC）中产生的。iPSC系携带FBN1基因突变，显示出正常的核型，表达了多能性标记，并具有在体内分化为三个胚层的能力。iPS系ZZUSAHi003-A可作为研究MFS致病机制的有用工具。