Abstract：
:We established three iPSC lines from postmortem-cultured fibroblasts derived following the sudden unexpected death of an 8-year-old girl with Lennox-Gastaut syndrome, who turned out to have the R551H-mutant STXBP1 gene. These iPSC clones showed pluripotent characteristics while retaining the genotype and demonstrated trilineage differentiation capability, indicating their utility in disease-modeling studies, i.e., STXBP1-encephalopathy. This is the first report on the establishment of iPSCs from a sudden death child, suggesting the possible use of postmortem-iPSC technologies as an epoch-making approach for precise identification of the cause of sudden death.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Yamamoto T,Otsu M,Okumura T,Horie Y,Ueno Y,Taniguchi H,Ohtaka M,Nakanishi M,Abe Y,Murase T,Umehara T,Ikematsu Kdoi
10.1016/j.scr.2019.101485subject
Has Abstractpub_date
2019-08-01 00:00:00pages
101485eissn
1873-5061issn
1876-7753pii
S1873-5061(19)30115-1journal_volume
39pub_type
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