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...

Descripción completa

Detalles Bibliográficos

Autores principales:	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
Formato:	Artigo
Lenguaje:	Inglês
Publicado:	Frontiers Media S.A. 2019-03-01
Colección:	Frontiers in Immunology
Materias:	LUBAC HOIP HOIL1 SHARPIN primary immunodeficiency autoinflammation
Acceso en línea:	https://www.frontiersin.org/article/10.3389/fimmu.2019.00479/full
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!