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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...
Autores principales: | Damgaard, Rune Busk, Elliott, Paul R, Swatek, Kirby N, Maher, Eamonn R, Stepensky, Polina, Elpeleg, Orly, Komander, David, Berkun, Yackov |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6404114/ https://www.ncbi.nlm.nih.gov/pubmed/30804083 http://dx.doi.org/10.15252/emmm.201809324 |
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