Article
Mutations affecting the actin regulator WD repeat-containing protein 1 lead to aberrant lymphoid immunity.
The Journal of allergy and clinical immunology - 1 Nov 2018
Pfajfer Laurène, Mair Nina K, Jiménez-Heredia Raúl, Genel Ferah, Gulez Nesrin, Ardeniz Ömür, Hoeger Birgit, Bal Sevgi Köstel, Madritsch Christoph, Kalinichenko Artem, Chandra Ardy Rico, Gerçeker Bengü, Rey-Barroso Javier, Ijspeert Hanna, Tangye Stuart G, Simonitsch-Klupp Ingrid, Huppa Johannes B, van der Burg Mirjam, Dupré Loïc, Boztug Kaan
Abstract excerpt
BACKGROUND: The actin-interacting protein WD repeat-containing protein 1 (WDR1) promotes cofilin-dependent actin filament turnover. Biallelic WDR1 mutations have been identified recently in an immunodeficiency/autoinflammatory syndrome with aberrant morphology and function of myeloid cells. OBJECTIVE: Given the pleiotropic expression of WDR1, here we investigated to what extent it might control the lymphoid arm...
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