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Immunodeficiency 56

Disease ID: disease_node_13313

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DbxrefMIM:615207, ORDO:357329, UMLS_CUI:C3554687
SubclassofDOID_628, DOID_0050737
Data SourceDOID
SynonymsIL21R immunodeficiency, IMD56, combined immunodeficiency due to IL21R deficiency
Doid Labelimmunodeficiency 56
Doid DescriptionA combined T cell and B cell immunodeficiency characterized by B- and T-cell defects including defective class-switched B cells, low IgG, defective antibody response, and defective T-cell responses to certain antigens, and variable dysfunction of NK cells that has_material_basis_in homozygous or compound heterozygous mutation in the IL21R gene on chromosome 16p12.1.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13313
Doid IdDOID_0111982
LabelImmunodeficiency 56