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Generation of 3 patient induced Pluripotent stem cell lines containing SORD mutations linked to a recessive neuropathy
Journal article   Open access   Peer reviewed

Generation of 3 patient induced Pluripotent stem cell lines containing SORD mutations linked to a recessive neuropathy

Christopher Yanick, Renata Maciel, Elizabeth Jacobs, Jacquelyn Schatzman, Michael Shy, Stephan Zuchner and Mario Saporta
Stem cell research, Vol.78, 103449
08/2024
DOI: 10.1016/j.scr.2024.103449
PMCID: PMC11259078
PMID: 38796985
url
https://doi.org/10.1016/j.scr.2024.103449View
Published (Version of record) Open Access

Abstract

The SORD neuropathy has been identified as the most common autosomal recessive inherited neuropathy, occurring in thousands of patients worldwide. Fibroblast lines from 3 different patients containing the c.753delG; p.Ala253GlnfsTer27 SORD mutations were reprogrammed into induced Pluripotent Stem Cell (iPSC) lines. These iPSC lines demonstrate an apparent normal karyotype and have positive expression of pluripotency markers. These iPSC lines also stain positively for Ectoderm, Endoderm and Mesoderm markers following Embryoid body differentiation. These lines pose to serve as a valuable disease modeling resource for studying the SORD neuropathy, including studying disease phenotype and treatment efficacy.

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