SHARPIN forms a linear ubiquitin ligase complex regulating NF-κB activity and apoptosis

@article{Ikeda2011SHARPINFA,
  title={SHARPIN forms a linear ubiquitin ligase complex regulating NF-κB activity and apoptosis},
  author={Fumiyo Ikeda and Yonathan Lissanu Deribe and Sigrid Strand Sk{\aa}nland and Benjamin Stieglitz and Caroline Grabbe and Mirita Franz-Wachtel and Sjoerd J. L. van Wijk and Panchali Goswami and Vanja Nagy and Jano{\vs} Terzi{\'c} and Fuminori Tokunaga and Ariadne Androulidaki and Tomoko Nakagawa and Manolis Pasparakis and Kazuhiro Iwai and John P. Sundberg and Liliana Schaefer and Katrin Rittinger and Boris Ma{\vc}ek and Ivan Dikic},
  journal={Nature},
  year={2011},
  volume={471},
  pages={637-641}
}
SHARPIN is a ubiquitin-binding and ubiquitin-like-domain-containing protein which, when mutated in mice, results in immune system disorders and multi-organ inflammation. Here we report that SHARPIN functions as a novel component of the linear ubiquitin chain assembly complex (LUBAC) and that the absence of SHARPIN causes dysregulation of NF-κB and apoptotic signalling pathways, explaining the severe phenotypes displayed by chronic proliferative dermatitis (cpdm) in SHARPIN-deficient mice. Upon… CONTINUE READING
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