原发性侧索硬化症(PLS)患者两种多能干细胞系的产生和特性分析

IF 0.7 4区 医学 Q4 BIOTECHNOLOGY & APPLIED MICROBIOLOGY
Laverde-Paz Mayra Juliana , Maki Kassidy , Melo-Escobar Isabel , McCartan Robyn , Benatar Michael , Wuu Joanne , Zeier Zane
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引用次数: 0

摘要

原发性侧索硬化(PLS)是一种极其罕见的疾病,其特征是上运动神经元的选择性变性。在这里,我们报告了两个人类诱导多能干细胞(iPSC)系的产生和验证,这些系来自不相关的男性和女性供体,没有任何已知的PLS家族史或可识别的遗传风险因素。除了少年亚型外,PLS主要是特发性的,现有的动物模型无法复制其临床特征。因此,这些iPSC系为研究散发的、成人发病的pls(最普遍的疾病形式)提供了宝贵的资源。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Generation and characterization of two pluripotent stem cell lines from Primary Lateral Sclerosis (PLS) patients
Primary Lateral Sclerosis (PLS) is an ultra-rare disorder characterized by the selective degeneration of upper motor neurons. Here, we report the generation and validation of two human induced pluripotent stem cell (iPSC) lines derived from unrelated male and female donors without any known family history of PLS or identifiable genetic risk factors. Aside from a juvenile subtype, PLS is predominantly idiopathic, and existing animal models fail to replicate its clinical features. As such, these iPSC lines offer a valuable resource for studying sporadic, adult-onset PLS—the most prevalent form of the disease.
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来源期刊
Stem cell research
Stem cell research 生物-生物工程与应用微生物
CiteScore
2.20
自引率
8.30%
发文量
338
审稿时长
55 days
期刊介绍: Stem Cell Research is dedicated to publishing high-quality manuscripts focusing on the biology and applications of stem cell research. Submissions to Stem Cell Research, may cover all aspects of stem cells, including embryonic stem cells, tissue-specific stem cells, cancer stem cells, developmental studies, stem cell genomes, and translational research. Stem Cell Research publishes 6 issues a year.
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