Clinical, immunological features and follow up of 20 patients with dedicator of cytokinesis 8 (DOCK8) deficiency
Sign Up to like & getrecommendations! Published in 2020 at "Pediatric Allergy and Immunology"
DOI: 10.1111/pai.13236
Abstract: Biallelic mutations in the dedicator of cytokinesis 8 gene (DOCK8) cause a progressive combined immunodeficiency (CID) characterized by susceptibility to severe viral skin infections, atopic diseases, recurrent respiratory infections, and malignancy. Hematopoietic stem cell transplantation… read more here.
Keywords: dock8 deficiency; dedicator cytokinesis; hsct; dock8 ... See more keywords
DOCK8 is essential for LFA-1 dependent positioning of T follicular helper cells in germinal centers.
Sign Up to like & getrecommendations! Published in 2020 at "JCI insight"
DOI: 10.1172/jci.insight.134508
Abstract: T follicular helper (Tfh) cell migration into germinal centers (GC) is essential for the generation of GC B cells and antibody responses to T dependent (TD) antigens. This process requires interactions between LFA-1 on Tfh… read more here.
Keywords: dependent positioning; germinal centers; follicular helper; antibody responses ... See more keywords
DOCK8 enforces immunological tolerance by promoting IL-2 signaling and immune synapse formation in Tregs.
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DOI: 10.1172/jci.insight.94298
Abstract: Patients deficient in the guanine nucleotide exchange factor DOCK8 have decreased numbers and impaired in vitro function of Tregs and make autoantibodies, but they seldom develop autoimmunity. We show that, similarly, Dock8-/- mice have decreased… read more here.
Keywords: immune synapse; tregs; immunological tolerance; enforces immunological ... See more keywords
Case Report: Hypomorphic Function and Somatic Reversion in DOCK8 Deficiency in One Patient With Two Novel Variants and Sclerosing Cholangitis
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DOI: 10.3389/fimmu.2021.673487
Abstract: DOCK8 deficiency is a combined immunodeficiency due to biallelic variants in dedicator of cytokinesis 8 (DOCK8) gene. The disease has a wide clinical spectrum encompassing recurrent infections (candidiasis, viral and bacterial infections), virally driven malignancies… read more here.
Keywords: reversion dock8; somatic reversion; function somatic; hypomorphic function ... See more keywords
Deciphering the role of DOCK8 in tumorigenesis by regulating immunity and the application of nanotechnology in DOCK8 deficiency therapy
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2022.1065029
Abstract: The dedicator of cytokinesis 8 (DOCK8) immunodeficiency syndrome is a severe immune disorder and characterized by serum IgE levels elevation, fungal and viral infections, dermatitis and food allergies. It was well known that DOCK8 is… read more here.
Keywords: regulating immunity; nanotechnology; role dock8; dock8 ... See more keywords