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Immunodeficiency 26

Disease ID: disease_node_13317

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DbxrefMIM:615966, NCI:C176795, ORDO:317425, UMLS_CUI:C4014833
SubclassofDOID_0050737, DOID_627
Data SourceDOID
SynonymsIMD26, SCID due to DNA-PKcs deficiency, immunodeficiency 26, with or without neurologic abnormalities, severe combined immunodeficiency due to DNA-PKcs deficiency
Doid Labelimmunodeficiency 26
Doid DescriptionA severe combined immunodeficiency characterized by virtually absent peripheral B and T cells but normal numbers of NK cells, complete block in B-cell differentiation, and a defect in slow repair of DNA double-strand breaks in fibroblasts, that has_material_basis_in homozygous or compound heterozygous mutation in the PRKDC gene on chromosome 8q11.21.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13317
Doid IdDOID_0111961
LabelImmunodeficiency 26