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The <jats:italic><jats:styled-content style=\"fixed-case\">DOCK<\/jats:styled-content>8<\/jats:italic> locus has many repetitive sequence elements that predispose to the generation of large germline deletions as well as recombination\u2010mediated somatic <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> repair. Residual <jats:styled-content style=\"fixed-case\">DOCK<\/jats:styled-content>8 protein contributes to the variable disease phenotype. The severe virus infections of the skin, and probably also <jats:styled-content style=\"fixed-case\">VZV<\/jats:styled-content>\u2010associated vasculopathy, reflect an important function of <jats:styled-content style=\"fixed-case\">DOCK<\/jats:styled-content>8, which is normally required to maintain lymphocyte shape integrity as the cells migrate through dense tissues. Loss of <jats:styled-content style=\"fixed-case\">DOCK<\/jats:styled-content>8 also causes immune deficits through other mechanisms including a milder generalized cell survival defect and skewing of T helper cell subsets. Recent work has uncovered the roles for <jats:styled-content style=\"fixed-case\">DOCK<\/jats:styled-content>8 in dendritic cell responses that can also help explain the virus susceptibility, as well as in regulatory T cells that might help explain autoimmunity in a minority of patients. Fortunately, hematopoietic stem cell transplantation cures the eczema and infection susceptibility of <jats:styled-content style=\"fixed-case\">DIDS<\/jats:styled-content>, but not necessarily the other disease manifestations including food allergies.<\/jats:p>","DOI":"10.1111\/imr.12723","type":"journal-article","created":{"date-parts":[[2018,12,19]],"date-time":"2018-12-19T01:12:24Z","timestamp":1545181944000},"page":"9-19","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":75,"title":["Insights into immunity from clinical and basic science studies of <scp>DOCK<\/scp>8 immunodeficiency syndrome"],"prefix":"10.1111","volume":"287","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5582-9110","authenticated-orcid":false,"given":"Helen C.","family":"Su","sequence":"first","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases National Institutes of Health  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