Induced Pluripotent Stem Cells from Individuals with Recessive Dystrophic Epidermolysis Bullosa
نویسندگان
چکیده
منابع مشابه
Generation of keratinocytes from normal and recessive dystrophic epidermolysis bullosa-induced pluripotent stem cells.
Embryonic stem cells (ESCs) have an unlimited proliferative capacity and extensive differentiation capability. They are an alternative source for regenerative therapies with a potential role in the treatment of several human diseases. The clinical use of ESCs, however, has significant ethical and biological obstacles related to their derivation from embryos and potential for immunological rejec...
متن کاملHuman COL7A1-corrected induced pluripotent stem cells for the treatment of recessive dystrophic epidermolysis bullosa.
Patients with recessive dystrophic epidermolysis bullosa (RDEB) lack functional type VII collagen owing to mutations in the gene COL7A1 and suffer severe blistering and chronic wounds that ultimately lead to infection and development of lethal squamous cell carcinoma. The discovery of induced pluripotent stem cells (iPSCs) and the ability to edit the genome bring the possibility to provide defi...
متن کاملPatient-specific naturally gene-reverted induced pluripotent stem cells in recessive dystrophic epidermolysis bullosa
Spontaneous reversion of disease-causing mutations has been observed in some genetic disorders. In our clinical observations of severe generalized recessive dystrophic epidermolysis bullosa (RDEB), a currently incurable blistering genodermatosis caused by loss-of-function mutations in COL7A1 that results in a deficit of type VII collagen (C7), we have observed patches of healthy-appearing skin ...
متن کاملThree Brothers with Recessive Dystrophic Epidermolysis Bullosa
Epidermolysis bullosa (EB) is a group of heritable mechano-bullous skin diseases classified into three major categories on the basis of the level of tissue separation within the dermal-epidermal basement membrane zone. In the most severe, dystrophic (scarring) forms of EB, blisters form below the cutaneous basement membrane at the level of the anchoring fibrils, which are composed of type VII c...
متن کاملKeratinocytes from Induced Pluripotent Stem Cells in Junctional Epidermolysis Bullosa
The skin biopsy was cut into 2 × 2 mm pieces. The dermis was peeled off and trapped under a sterile cover slip. Human fibroblast media consisted of DMEM (Invitrogen, Carlsbad, CA), 10% fetal bovine serum (Invitrogen), and 1% penicillin/streptomycin (Invitrogen). Medium was changed every 2 days until the culture became 90% confluent, at which point it was passaged using 0.25% trypsin EDTA. Human...
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ژورنال
عنوان ژورنال: Journal of Investigative Dermatology
سال: 2011
ISSN: 0022-202X
DOI: 10.1038/jid.2010.346