Dataset Information


Modeling the pathogenesis of Charcot-Marie-Tooth disease type 1A using patient-specific iPSCs

ABSTRACT: Here, human induced pluripotent stem cells (control-hiPSCs, CMT1A-hiPSCs, and PMP22-hiPSCs) were induced to differentiate to Schwann cells (control-SCs, CMT1A-SCs, and PMP22-SCs) through neural crest stage (control-NCSCs, CMT1A-NCSCs, and PMP22-NCSCs). We sequenced mRNA samples from Schwann cell differentiation of human pluripotent stem cells at 3 different stage to generate the gene expression profiles of these cells. Overall design: 7 samples, including undifferentiated hiPSCs (control-hiPSCs and CMT1A-hiPSCs), freshly isolated p75+/HNK1+ NCSCs (control-NCSCs and CMT1A-NCSCs), and SCs (control-SCs, CMT1A-SCs, and PMP22-SCs) were analyzed.

INSTRUMENT(S): Illumina Genome Analyzer (Homo sapiens)

SUBMITTER: Weiqiang Li  

PROVIDER: GSE97851 | GEO | 2018-02-14


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GSE97851_RNA-seq_data_of_SCs.xlsx Xlsx
GSE97851_RNA-seq_data_of_hiPSCs_and_NCSCs.xlsx Xlsx
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Charcot-Marie-Tooth disease type 1A (CMT1A), one of the most frequent inherited peripheral neuropathies, is associated with PMP22 gene duplication. Previous studies of CMT1A mainly relied on rodent models, and it is not yet clear how PMP22 overexpression leads to the phenotype in patients. Here, we generated the human induced pluripotent stem cell (hiPSC) lines from two CMT1A patients as an in vitro cell model. We found that, unlike the normal control cells, CMT1A hiPSCs rarely generated Schwann  ...[more]

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