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

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

Abstract 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...

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Bibliographic Details
Main Authors: Rune Busk Damgaard, Paul R Elliott, Kirby N Swatek, Eamonn R Maher, Polina Stepensky, Orly Elpeleg, David Komander, Yackov Berkun
Format: Article
Language:English
Published: Springer Nature 2019-02-01
Series:EMBO Molecular Medicine
Subjects:
cell death
deubiquitinases
inflammatory disease
TNF signalling
ubiquitin
Online Access:https://doi.org/10.15252/emmm.201809324
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https://doi.org/10.15252/emmm.201809324

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