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Author Details

Douglas B Kuhns
Frederick National Laboratory for Cancer Research
1984
120
54
PMIDPaper TitleJournal TitlePublished Year
37488949Anakinra-Associated Systemic Amyloidosis.Arthritis Rheumatol2024
36378426A Heterozygous Gain-of-Function Variant in IKBKB Associated with Autoimmunity and Autoinflammation.J Clin Immunol2023
37561579A phase III randomized crossover trial of plerixafor versus G-CSF for treatment of WHIM syndrome.J Clin Invest2023
37824621Characterization of the antispike IgG immune response to COVID-19 vaccines in people with a wide variety of immunodeficiencies.Sci Adv2023
37349293Inherited ARPC5 mutations cause an actinopathy impairing cell motility and disrupting cytokine signaling.Nat Commun2023
36696755Hematologically important mutations: Leukocyte adhesion deficiency (second update).Blood Cells Mol Dis2023
36932076Constitutively active Lyn kinase causes a cutaneous small vessel vasculitis and liver fibrosis syndrome.Nat Commun2023
35177862Immunopathological signatures in multisystem inflammatory syndrome in children and pediatric COVID-19.Nat Med2022
35344128Interpretation of Dihydrorhodamine-1,2,3 Flow Cytometry in Chronic Granulomatous Disease: an Atypical Exemplar.J Clin Immunol2022
35852866Multicenter analysis of neutrophil extracellular trap dysregulation in adult and pediatric COVID-19.JCI Insight2022
35868845Gain-of-function mutations in <i>ALPK1</i> cause an NF-κB-mediated autoinflammatory disease: functional assessment, clinical phenotyping and disease course of patients with ROSAH syndrome.Ann Rheum Dis2022
36166305Immunogenetics associated with severe coccidioidomycosis.JCI Insight2022
36094106Preclinical Evaluation of Foamy Virus Vector-Mediated Gene Addition in Human Hematopoietic Stem/Progenitor Cells for Correction of Leukocyte Adhesion Deficiency Type 1.Hum Gene Ther2022
36377664Human Dectin-1 deficiency impairs macrophage-mediated defense against phaeohyphomycosis.J Clin Invest2022
32886268Low Plasma Gelsolin Concentrations in Chronic Granulomatous Disease.Inflammation2021
33623984Enhanced homology-directed repair for highly efficient gene editing in hematopoietic stem/progenitor cells.Blood2021
33876203SASH3 variants cause a novel form of X-linked combined immunodeficiency with immune dysregulation.Blood2021
33785481Phase I Study of Zotiraciclib in Combination with Temozolomide for Patients with Recurrent High-grade Astrocytomas.Clin Cancer Res2021
33892719Homozygous variant p. Arg90His in NCF1 is associated with early-onset Interferonopathy: a case report.Pediatr Rheumatol Online J2021
34547651Hematologically important mutations: The autosomal forms of chronic granulomatous disease (third update).Blood Cells Mol Dis2021
34175765Hematologically important mutations: X-linked chronic granulomatous disease (fourth update).Blood Cells Mol Dis2021
33232303An immune-based biomarker signature is associated with mortality in COVID-19 patients.JCI Insight2021
32311393Cutaneous barrier leakage and gut inflammation drive skin disease in Omenn syndrome.J Allergy Clin Immunol2020
33284949NADPH oxidase correction by mRNA transfection of apheresis granulocytes in chronic granulomatous disease.Blood Adv2020
30651282<i>NCF1</i> (p47<sup>phox</sup>)-deficient chronic granulomatous disease: comprehensive genetic and flow cytometric analysis.Blood Adv2019
31807607Patients with Idiopathic Pulmonary Nontuberculous Mycobacterial Disease Have Normal Th1/Th2 Cytokine Responses but Diminished Th17 Cytokine and Enhanced Granulocyte-Macrophage Colony-Stimulating Factor Production.Open Forum Infect Dis2019
31554793Human PI3Kγ deficiency and its microbiota-dependent mouse model reveal immunodeficiency and tissue immunopathology.Nat Commun2019
31210423Neutrophil activation in systemic capillary leak syndrome (Clarkson disease).J Cell Mol Med2019
31167928Lymphocyte-driven regional immunopathology in pneumonitis caused by impaired central immune tolerance.Sci Transl Med2019
30723080Dominant activating RAC2 mutation with lymphopenia, immunodeficiency, and cytoskeletal defects.Blood2019
31172495Diagnostic Testing for Chronic Granulomatous Disease.Methods Mol Biol2019
28528201X-linked carriers of chronic granulomatous disease: Illness, lyonization, and stability.J Allergy Clin Immunol2018
29969437Inherited p40phox deficiency differs from classic chronic granulomatous disease.J Clin Invest2018
29856956Genetic Inactivation of CD33 in Hematopoietic Stem Cells to Enable CAR T Cell Immunotherapy for Acute Myeloid Leukemia.Cell2018
30035749TNF overproduction impairs epithelial staphylococcal response in hyper IgE syndrome.J Clin Invest2018
29534156IKBKG (NEMO) 5' Untranslated Splice Mutations Lead to Severe, Chronic Disseminated Mycobacterial Infections.Clin Infect Dis2018
29145578The Changing Paradigm of Management of Liver Abscesses in Chronic Granulomatous Disease.Clin Infect Dis2018
28752258Allogeneic Reduced-Intensity Hematopoietic Stem Cell Transplantation for Chronic Granulomatous Disease: a Single-Center Prospective Trial.J Clin Immunol2017
28077679CRISPR-Cas9 gene repair of hematopoietic stem cells from patients with X-linked chronic granulomatous disease.Sci Transl Med2017
28240310Interleukin-27 Enhances the Potential of Reactive Oxygen Species Generation from Monocyte-derived Macrophages and Dendritic cells by Induction of p47<sup>phox</sup>.Sci Rep2017
29234000Peritoneal tissue-resident macrophages are metabolically poised to engage microbes using tissue-niche fuels.Nat Commun2017
27099176Lentiviral hematopoietic stem cell gene therapy for X-linked severe combined immunodeficiency.Sci Transl Med2016
29296942Gene-edited pseudogene resurrection corrects p47<sup>phox</sup>-deficient chronic granulomatous disease.Blood Adv2016
26545803Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy.Clin Gastroenterol Hepatol2016
27861181Genetic Risk for Inflammatory Bowel Disease Is a Determinant of Crohn's Disease Development in Chronic Granulomatous Disease.Inflamm Bowel Dis2016
27301573Complete Myeloperoxidase Deficiency: Beware the "False-Positive" Dihydrorhodamine Oxidation.J Pediatr2016
27557945Cytoskeletal abnormalities and neutrophil dysfunction in WDR1 deficiency.Blood2016
27044504Colitis susceptibility in p47(phox-/-) mice is mediated by the microbiome.Microbiome2016
25288370An AAVS1-targeted minigene platform for correction of iPSCs from all five types of chronic granulomatous disease.Mol Ther2015
26528633Isolation and Functional Analysis of Human Neutrophils.Curr Protoc Immunol2015
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Collaborators

Debra A Long Priel
Leidos Biomedical Research Inc., Frederick National Laboratory for Cancer Research
Co-authored papers 33
Alexandra F Freeman
National Institute of Allergy and Infectious Diseases
Co-authored papers 15
Stefania Pittaluga
National Cancer Institute, National Institutes of Health
Co-authored papers 12
Sergio D Rosenzweig
National Institute of Allergy and Infectious Diseases
Co-authored papers 11
Theo Heller
National Institute of Diabetes and Digestive and Kidney Diseases
Co-authored papers 9
Edward W Cowen
National Institute of Arthritis and Musculoskeletal and Skin Diseases
Co-authored papers 7
Joshua D Milner
Columbia University
Co-authored papers 5
Xiaolin Wu
Leidos Biomedical Research Inc.
Co-authored papers 5
Karen Lau
Inc., Frederick National Laboratory for Cancer Research
Co-authored papers 5
Daniel W McVicar
National Cancer Institute
Co-authored papers 5
Joie Davis
National Institutes of Health
Co-authored papers 4
Daniel L Kastner
Co-authored papers 4
Jean K Lim
Icahn School of Medicine at Mount Sinai
Co-authored papers 3
Jenna R E Bergerson
National Institute of Allergy and Infectious Diseases, National Institutes of Health
Co-authored papers 3
Mark Raffeld
Center for Cancer Research, National Cancer Institute
Co-authored papers 3
Benjamin D Greenberg
Warren Alpert School of Medicine, Brown University, Butler Hospital
Co-authored papers 2
Massimo Gadina
National Institute of Arthritis and Musculoskeletal and Skin Diseases
Co-authored papers 2
Javed Khan
Center for Cancer Research, National Cancer Institute
Co-authored papers 2
Clifton L Dalgard
Uniformed Services University of the Health Sciences
Co-authored papers 2
Rhonda Brandon
University of Queensland
Co-authored papers 2
Stephen M Hewitt
Center for Cancer Research, National Cancer Institute
Co-authored papers 2
Marcus Y Chen
and Blood Institute, National Institutes of Health
Co-authored papers 2
Dirk Foell
Co-authored papers 2
Wanxia Li Tsai
National Institute of Arthritis
Co-authored papers 2
Sandra Burkett
Center for Cancer Research, National Cancer Institute, National Institutes of Health
Co-authored papers 2
Christoph Klein
Kinderklinik und Kinderpoliklinik der Ludwig-Maximilians-Universitat Munchen
Co-authored papers 1
Patricia A Peyser
School of Public Health, University of Michigan ann arbor
Co-authored papers 1
Michele Nehrebecky
National Human Genome Research Institute, National Institutes of Health (NIH)
Co-authored papers 1
Mauno Vihinen
Lund University
Co-authored papers 1
Jacqueline W Mays
National Institute of Dental and Craniofacial Research, National Institutes of Health
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Release 1.2