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Lesch-Nyhan Syndrome

Disease ID: disease_node_4642

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DbxrefICD10CM:E79.1, MESH:D007926, MIM:300322, NCI:C61255, SNOMEDCT_US_2023_03_01:190918000, UMLS_CUI:C0023374
SubclassofDOID_0080012, DOID_653
Data SourceDOID, MESH
SynonymsComplete hypoxanthine-guanine phosphoribosyltransferase deficiency, HG-PRT deficiency, HPRT1 deficiency, Hypoxanthine-guanine phosphoribosyltransferase deficiency, Hypoxanthine-guanine-phosphoribosyltransferase deficiency, Lesch - Nyhan syndrome, X-linked hyperuricemia, deficiency of IMP pyrophosphorylase, hypoxanthine guanine phosphoribosyltransferase deficiency
Mesh IdD007926
Mesh LabelLesch-Nyhan Syndrome
Mesh SubclassofD038901, D020739, D011686
Doid LabelLesch-Nyhan syndrome
Doid DescriptionA purine-pyrimidine metabolic disorder characterized by mental retardation, spastic cerebral palsy, choreoathetosis, uric acid urinary stones, and self-destructive biting of fingers and lips that has_material_basis_in mutation in the HPRT1 gene on chromosome Xq26. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000149
Disease Node Iddisease_node_4642
Doid IdDOID_1919
LabelLesch-Nyhan Syndrome