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HOIL and water: the two faces of HOIL-1 deficiency

Nature Immunology volume 13, pages 1133–1135 (2012)Cite this article

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Autoinflammation and immunodeficiency are rare in humans, but the rate of discovery of these conditions has increased. Three patients have now been characterized in whom deficiency in HOIL-1, a component of the LUBAC complex, leads to these conditions.

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Figure 1: Different effects of HOIL-1 deficiency in lymphocytes and monocytes.

Debbie Maizels

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Authors and Affiliations

  1. Michael J. Ombrello and Daniel L. Kastner are with the Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.,

    Michael J Ombrello & Daniel L Kastner

  2. Joshua D. Milner is with the Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA.,

    Joshua D Milner

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  1. Michael J Ombrello
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  2. Daniel L Kastner
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  3. Joshua D Milner
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Correspondence to Michael J Ombrello or Daniel L Kastner.

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Ombrello, M., Kastner, D. & Milner, J. HOIL and water: the two faces of HOIL-1 deficiency. Nat Immunol 13, 1133–1135 (2012). https://doi.org/10.1038/ni.2471

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