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Combined Immunodeficiency Associated with DOCK8 Mutations and Related Immunodeficiencies
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The Essential Role of DOCK8 in Humoral Immunity
Katrina L. Randall,1,2 Teresa Lambe,3 Chris C. Goodnow,1 and Richard J. Cornall
31John Curtin School of Medical Research, The Australian National University, Canberra ACT, Australia
2Department of Immunology, The Canberra Hospital, Garran, ACT, Australia
3Nuffield Department of Clinical Medicine, Oxford University, UK
Abstract
The processes that normally generate and maintain adaptive immunity and immunological memory are poorly understood, and yet of fundamental importance when infectious diseases place such a major economic and social burden on the world's health and agriculture systems. Defects in these mechanisms also underlie the many forms of human primary immunodeficiency. Identifying these mechanisms in a systematic way is therefore important if we are to develop better strategies for treating and preventing infection, inherited disease, transplant rejection and autoimmunity. In this review we describe a genome-wide screen in mice for the genes important for generating these adaptive responses, and describe two independent DOCK8 mutant mice strains identified by this screen. DOCK 8 was found to play an essential role in humoral immune responses and to be important in the proper formation of the B cell immunological synapse.
Copyright
Copyright © 2010 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
