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

  title={HOIP deficiency causes embryonic lethality by aberrant TNFR1-mediated endothelial cell death.},
  author={N. Peltzer and E. Rieser and L. Taraborrelli and P. Draber and Maurice Darding and B{\'a}rbara Pernaute and Y. Shimizu and Aida Sarr and Helena Draberova and A. Montinaro and J. P. Martinez-Barbera and J. Silke and T. Rodr{\'i}guez and H. Walczak},
  journal={Cell reports},
  volume={9 1},
  • N. Peltzer, E. Rieser, +11 authors H. Walczak
  • Published 2014
  • Medicine, Biology
  • Cell reports
  • 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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