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X-Linked Combined Immunodeficiency Diseases

Disease ID: disease_node_11755

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DbxrefGARD:5618, MESH:D053632, MIM:300400, NCI:C4682, SNOMEDCT_US_2023_03_01:203592006, UMLS_CUI:C1279481
SubclassofDOID_0080012, DOID_627
Data SourceDOID, MESH
SynonymsSCID-X1, XSCID, gamma chain deficiency, thymic epithelial hypoplasia
Mesh IdD053632
Mesh LabelX-Linked Combined Immunodeficiency Diseases
Mesh SubclassofD040181, D016511
Doid LabelX-linked severe combined immunodeficiency
Doid DescriptionA severe combined immunodeficiency that is a X-linked SCID that has_material_basis_in mutations in genes encoding common gamma chain proteins shared by the interleukin (IL-2,4,7,9,16 and21) receptors resulting in a non-functional gamma chain, defective interleukin signalling, minimal or ascent T- and NK cells and non-functional B-cells. OMIM mapping confirmed by DO. [LS].
Has Material Basis InGENO_0000149
Disease Node Iddisease_node_11755
Doid IdDOID_0060013
LabelX-Linked Combined Immunodeficiency Diseases
Doid Alternate IdsDOID_5811