Intrinsic Defects in B Cell Development and Differentiation, T Cell Exhaustion and Altered Unconventional T Cell Generation Characterize Human Adenosine Deaminase Type 2 Deficiency

Purpose Deficiency of adenosine deaminase type 2 (ADA2) (DADA2) is a rare inborn error of immunity caused by deleterious biallelic mutations in ADA2 . Clinical manifestations are diverse, ranging from severe vasculopathy with lacunar strokes to immunodeficiency with viral infections, hypogammaglobul...

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Bibliographic Details
Published in:	Journal of clinical immunology Vol. 41; no. 8; pp. 1915 - 1935
Main Authors:	Yap, Jin Yan, Moens, Leen, Lin, Ming-Wei, Kane, Alisa, Kelleher, Anthony, Toong, Catherine, Wu, Kathy H.C., Sewell, William A., Phan, Tri Giang, Hollway, Georgina E., Enthoven, Karen, Gray, Paul E., Casas-Martin, Jose, Wouters, Carine, De Somer, Lien, Hershfield, Michael, Bucciol, Giorgia, Delafontaine, Selket, Ma, Cindy S., Tangye, Stuart G., Meyts, Isabelle
Format:	Journal Article
Language:	English
Published:	New York Springer US 01.11.2021 Springer Nature B.V
Subjects:	Adenosine Adenosine deaminase Adenosine Deaminase - blood Adenosine Deaminase - deficiency Adenosine Deaminase - genetics Adolescent Adult Agammaglobulinemia - blood Agammaglobulinemia - genetics Agammaglobulinemia - immunology Aged B-Lymphocytes - immunology Biomedical and Life Sciences Biomedicine Bone marrow CD4 antigen CD8 antigen Cell Differentiation Child Child, Preschool Dendritic Cells - immunology Etanercept Genotype & phenotype Humans Hypogammaglobulinemia Immunodeficiency Immunological memory Immunology Infant Infectious Diseases Inflammation Intercellular Signaling Peptides and Proteins - blood Intercellular Signaling Peptides and Proteins - deficiency Intercellular Signaling Peptides and Proteins - genetics Interferon Internal Medicine Killer Cells, Natural - immunology Leukocytes Lymphocytes Lymphocytes B Lymphocytes T Medical Microbiology Memory cells Middle Aged Monocytes Mutation Original Original Article Patients Phenotypes Senescence Severe Combined Immunodeficiency - blood Severe Combined Immunodeficiency - genetics Severe Combined Immunodeficiency - immunology Steroid hormones T-Lymphocytes - immunology Vascular diseases Viral infections Young Adult SIGLEC-1 Monocytes DADA2 Type I IFN signature T cell exhaustion Humoral immunodeficiency ADA2 deficiency
ISSN:	0271-9142, 1573-2592, 1573-2592
Online Access:	Get full text
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