Skin lesion development in a mouse model of incontinentia pigmenti is triggered by NEMO deficiency in epidermal keratinocytes and requires TNF signaling.

@article{Nenci2006SkinLD,
  title={Skin lesion development in a mouse model of incontinentia pigmenti is triggered by NEMO deficiency in epidermal keratinocytes and requires TNF signaling.},
  author={Arianna Nenci and Marion Huth and Alfred Funteh and Marc Schmidt-Supprian and Wilhelm Bloch and Daniel Metzger and Pierre Chambon and K. Rajewsky and Thomas Krieg and Ingo Haase and Manolis Pasparakis},
  journal={Human molecular genetics},
  year={2006},
  volume={15 4},
  pages={531-42}
}
NF-kappaB essential modulator (NEMO), the regulatory subunit of the IkappaB kinase, is essential for NF-kappaB activation. Mutations disrupting the X-linked NEMO gene cause incontinentia pigmenti (IP), a human genetic disease characterized by male embryonic lethality and by a complex pathology affecting primarily the skin in heterozygous females. The cellular and molecular mechanisms leading to skin lesion pathogenesis in IP patients remain elusive. Here we used epidermis-specific deletion of… CONTINUE READING
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