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Stem Cell Res ; 75: 103306, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38271763

ABSTRACT

We have generated MLi005-A, a new induced pluripotent stem cell (iPSC) line derived from skin fibroblasts of a male patient with dominant dystrophic epidermolysis bullosa (DDEB). This iPSC line may be used as a model system for studies on skin integrity, the extracellular matrix and skin barrier function. The characterization of the MLi005-A cell line consisted of molecular karyotyping, next-generation sequencing of the COL7A1 alleles, pluripotency and differentiation potentials testing by immunofluorescence of associated markers in vitro. The MLi-005A line has been also tested for ability to differentiate into fibroblasts and keratinocytes and markers associated with these cell types.


Subject(s)
Epidermolysis Bullosa Dystrophica , Induced Pluripotent Stem Cells , Humans , Male , Epidermolysis Bullosa Dystrophica/genetics , Epidermolysis Bullosa Dystrophica/metabolism , Induced Pluripotent Stem Cells/metabolism , Collagen Type VII/genetics , Collagen Type VII/metabolism , Skin/metabolism , Keratinocytes/metabolism
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