OTULIN deficiency in ORAS causes cell type‐specific LUBAC degradation, dysregulated TNF signalling and cell death

The deubiquitinase OTULIN removes methionine‐1 (M1)‐linked polyubiquitin signals conjugated by the linear ubiquitin chain assembly complex (LUBAC) and is critical for preventing TNF‐driven inflammation in OTULIN‐related autoinflammatory syndrome (ORAS). Five ORAS patients have been reported, but how...

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Publicado en:EMBO Mol Med
Autores principales: Damgaard, Rune Busk, Elliott, Paul R, Swatek, Kirby N, Maher, Eamonn R, Stepensky, Polina, Elpeleg, Orly, Komander, David, Berkun, Yackov
Formato: Artigo
Lenguaje:Inglês
Publicado: John Wiley and Sons Inc. 2019
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Research Articles
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6404114/
https://ncbi.nlm.nih.gov/pubmed/30804083
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.15252/emmm.201809324
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