HOIP deficiency causes embryonic lethality by aberrant TNFR1-mediated endothelial cell death.

@article{Peltzer2014HOIPDC,
  title={HOIP deficiency causes embryonic lethality by aberrant TNFR1-mediated endothelial cell death.},
  author={Nieves Peltzer and Eva Rieser and Lucia Taraborrelli and Peter Dr{\'a}ber and Maurice Darding and B{\'a}rbara Pernaute and Yutaka Shimizu and Aida Sarr and Helena Draberova and Antonella Montinaro and Juan Pedro Martinez-Barbera and John Silke and Tristan A Rodr{\'i}guez and Henning Walczak},
  journal={Cell reports},
  year={2014},
  volume={9 1},
  pages={
          153-165
        }
}
Linear ubiquitination is crucial for innate and adaptive immunity. The linear ubiquitin chain assembly complex (LUBAC), consisting of HOIL-1, HOIP, and SHARPIN, is the only known ubiquitin ligase that generates linear ubiquitin linkages. HOIP is the catalytically active LUBAC component. Here, we show that both constitutive and Tie2-Cre-driven HOIP deletion lead to aberrant endothelial cell death, resulting in defective vascularization and embryonic lethality at midgestation. Ablation of tumor… CONTINUE READING
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