LUBAC is essential for embryogenesis by preventing cell death and enabling haematopoiesis

• Published in: Nature (London) Vol. 557; no. 7703; pp. 112 - 117
• Main Authors: Peltzer, Nieves, Darding, Maurice, Montinaro, Antonella, Draber, Peter, Draberova, Helena, Kupka, Sebastian, Rieser, Eva, Fisher, Amanda, Hutchinson, Ciaran, Taraborrelli, Lucia, Hartwig, Torsten, Lafont, Elodie, Haas, Tobias L., Shimizu, Yutaka, Böiers, Charlotta, Sarr, Aida, Rickard, James, Alvarez-Diaz, Silvia, Ashworth, Michael T., Beal, Allison, Enver, Tariq, Bertin, John, Kaiser, William, Strasser, Andreas, Silke, John, Bouillet, Philippe, Walczak, Henning
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.05.2018; Nature Publishing Group
• Subjects: 13; 13/1; 13/2; 13/21; 13/31; 13/51; 14; 14/1; 14/34; 14/63; 38; 38/91; 42; 45; 631/136/2086; 631/250/232/1473; 631/80/458/582; 631/80/82; 64; 64/60; 82; 82/1; Animals; Apoptosis; Assembly; Autoimmune diseases; Autoimmunity; Biochemistry; Carrier Proteins - chemistry; Carrier Proteins - genetics; Carrier Proteins - metabolism; Caspase; Caspase 8 - genetics; Caspase 8 - metabolism; Caspase-8; Cell activation; Cell death; Cell Death - genetics; Clonal deletion; Defects; Deregulation; Disease; Embryo Loss - genetics; Embryogenesis; Embryonic development; Embryonic Development - genetics; Embryonic growth stage; Embryos; Endothelial cells; Endothelial Cells - cytology; Endothelium; Female; Fetuses; Gestation; Health aspects; Hematopoiesis; Hematopoiesis - genetics; Humanities and Social Sciences; Inflammation; Kinases; Lethality; Letter; Ligases; Mice; Mice, Inbred C57BL; Mortality; multidisciplinary; Mutation; Phenotypes; Physiological aspects; Prevention; Protein Domains; Protein Kinases - genetics; Receptor-Interacting Protein Serine-Threonine Kinases - deficiency; Receptors; Receptors, Tumor Necrosis Factor, Type I - metabolism; Recruitment; Science; Science (multidisciplinary); Signal Transduction; Trends; Tumor necrosis factor receptors; Tumor necrosis factor-TNF; Ubiquitin; Ubiquitin - metabolism; Ubiquitin-Protein Ligases - deficiency; Ubiquitin-Protein Ligases - genetics; Ubiquitin-Protein Ligases - metabolism; United States > US

The linear ubiquitin chain assembly complex (LUBAC) is required for optimal gene activation and prevention of cell death upon activation of immune receptors, including TNFR1 1 . Deficiency in the LUBAC components SHARPIN or HOIP in mice results in severe inflammation in adulthood or embryonic lethal...