Article
Corrective Transduction of Human Epidermal Stem Cells in Laminin-5-Dependent Junctional Epidermolysis Bullosa
10 Jun 1998
Abstract excerpt
Laminin-5 is composed of three distinct polypeptides, α3, β3, and γ2, which are encoded by three different genes, LAMA3, LAMB3, and LAMC2, respectively. We have isolated epidermal keratinocytes from a patient presenting with a lethal form of junctional epidermolysis bullosa characterized by a homozygous mutation of the LAMB3 gene, which led to complete absence of the β3 polypeptide. In vitro, β3-null...
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