Corrective transduction of human epidermal stem cells in laminin-5-dependent junctional epidermolysis bullosa.

@article{Dellambra1998CorrectiveTO,
  title={Corrective transduction of human epidermal stem cells in laminin-5-dependent junctional epidermolysis bullosa.},
  author={Elena Dellambra and Jo{\"e}lle Vailly and Graziella Pellegrini and Sergio Bondanza and Osvaldo Golisano and Catia Addolorata Di Macchia and Giovanna Zambruno and Guerrino Meneguzzi and Michele De Luca},
  journal={Human gene therapy},
  year={1998},
  volume={9 9},
  pages={1359-70}
}
Laminin-5 is composed of three distinct polypeptides, alpha3, beta3, and gamma2, 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 beta3 polypeptide. In vitro, beta3-null keratinocytes were unable to synthesize laminin-5 and to assemble… CONTINUE READING

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