从一名携带GJB2 pV37I突变的台湾听力损失患者中生成诱导多能干细胞MMCi001-A。
Generation of induced pluripotent stem cells MMCi001-A from a Taiwanese hearing loss patient carrying GJB2 pV37I mutation.
作者信息
Lu Huai-En, Tsai Chia-Ling, Chiu Ing-Ming, Pan Yu-Ling, Lin Yi-Feng, Lin Hung-Ching, Hsu Yi-Chao
机构信息
Bioresource Collection and Research Center, Food Industry Research and Development Institute, Hsinchu, Taiwan.
Institute of Biomedical Sciences, Mackay Medical College, New Taipei City, Taiwan.
出版信息
Stem Cell Res. 2020 Jan;42:101692. doi: 10.1016/j.scr.2019.101692. Epub 2019 Dec 18.
Hearing loss is the most common disorder in the sensory system. Mutations in GJB2 have been reported to be very common in sensorineural hearing loss patients. In this report, we generated an induced pluripotent stem cell (iPSC) line, MMCi001-A, from the peripheral blood mononuclear cells of a 4-year-old male hearing loss patient carrying GJB2 pV37I mutation by using the Sendai virus delivery system. The generated iPSCs were demonstrated to express pluripotent markers and be differentiated into three germ layers in vitro and in vivo. This GJB2-pV37I iPSCs is valuable for studying the pathogenic mechanisms and drug discovery of hearing loss.
听力损失是感觉系统中最常见的疾病。据报道，GJB2基因的突变在感音神经性听力损失患者中非常普遍。在本报告中，我们使用仙台病毒递送系统，从一名携带GJB2 pV37I突变的4岁男性听力损失患者的外周血单个核细胞中生成了诱导多能干细胞（iPSC）系MMCi001-A。所生成的诱导多能干细胞被证明可表达多能性标志物，并在体外和体内分化为三个胚层。这种携带GJB2-pV37I突变的诱导多能干细胞对于研究听力损失的致病机制和药物研发具有重要价值。
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