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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Publication Details

Journal
Stem Cell Research
Published
2025-09-09
DOI
https://doi.org/10.1016/j.scr.2025.103828
Primary Topic
Amyotrophic Lateral Sclerosis Research
Type
article
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Generation and characterization of two pluripotent stem cell lines from Primary Lateral Sclerosis (PLS) patients

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Generation and characterization of two pluripotent stem cell lines from Primary Lateral Sclerosis (PLS) patients

María Isabel, Benedict Michael, Laverde-Paz Mayra Juliana, Maki Kassidy, McCartan Robyn, Wuu Joanne, Zeier Zane
article en

Abstract

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.

Stem Cell ResearchVol. 88
University of Miami (US), Sylvester Comprehensive Cancer Center (US)
Openalex Percentile: Top 65%
Amyotrophic Lateral Sclerosis Research
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