Second Case of HOIP Deficiency Expands Clinical Features and Defines Inflammatory Transcriptome Regulated by LUBAC

Background: HOIP is the catalytic subunit of the linear ubiquitination chain assembly complex (LUBAC) that is essential for NF-κB signaling and thus proper innate and adaptive immunity. To date only one patient with HOIP deficiency has been reported with clinical characteristics that include autoinf...

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Autori principali:	Hirotsugu Oda, David B. Beck, Hye Sun Kuehn, Natalia Sampaio Moura, Patrycja Hoffmann, Maria Ibarra, Jennifer Stoddard, Wanxia Li Tsai, Gustavo Gutierrez-Cruz, Massimo Gadina, Sergio D. Rosenzweig, Daniel L. Kastner, Luigi D. Notarangelo, Ivona Aksentijevich
Natura:	Artigo
Lingua:	Inglês
Pubblicazione:	Frontiers Media S.A. 2019-03-01
Serie:	Frontiers in Immunology
Soggetti:	LUBAC HOIP HOIL1 SHARPIN primary immunodeficiency autoinflammation
Accesso online:	https://www.frontiersin.org/article/10.3389/fimmu.2019.00479/full
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