{"title":"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","authors":"Laverde-Paz Mayra Juliana , Maki Kassidy , Melo-Escobar Isabel , McCartan Robyn , Benatar Michael , Wuu Joanne , Zeier Zane","doi":"10.1016/j.scr.2025.103828","DOIUrl":null,"url":null,"abstract":"<div><div>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.</div></div>","PeriodicalId":21843,"journal":{"name":"Stem cell research","volume":"88 ","pages":"Article 103828"},"PeriodicalIF":0.7000,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Generation and characterization of two pluripotent stem cell lines from Primary Lateral Sclerosis (PLS) patients\",\"authors\":\"Laverde-Paz Mayra Juliana , Maki Kassidy , Melo-Escobar Isabel , McCartan Robyn , Benatar Michael , Wuu Joanne , Zeier Zane\",\"doi\":\"10.1016/j.scr.2025.103828\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><div>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.</div></div>\",\"PeriodicalId\":21843,\"journal\":{\"name\":\"Stem cell research\",\"volume\":\"88 \",\"pages\":\"Article 103828\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2025-09-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Stem cell research\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1873506125001783\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"BIOTECHNOLOGY & APPLIED MICROBIOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Stem cell research","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1873506125001783","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"BIOTECHNOLOGY & APPLIED MICROBIOLOGY","Score":null,"Total":0}
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.
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.