Term Name:	immunodeficiency 26
Synonyms:	IMD26, immunodeficiency 26, with or without neurologic abnormalities, SCID due to DNA-PKcs deficiency, severe combined immunodeficiency due to DNA-PKcs deficiency
Definition:	A 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.
Ontology:	Human Disease [DOID:0111961]   ( DOID:0111961 )