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Immunodeficiency 47

Disease ID: disease_node_13252

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DbxrefMIM:300972, UMLS_CUI:C4310819
SubclassofDOID_0080012, DOID_0050571, DOID_612
Data SourceDOID
SynonymsCDG IIs, CDG2S, CDGIIs, IMD47, congenital disorder of glycosylation type IIs, immunodeficiency and hepatopathy with or without neurologic features
Doid Labelimmunodeficiency 47
Doid DescriptionA primary immunodeficiency disease characterized by liver dysfunction, recurrent bacterial infections, hypogammaglobulinemia, and defective glycosylation of serum proteins that has_material_basis_in hemizygous mutation in the ATP6AP1 gene on chromosome Xq28.
Has PhenotypeHP_0010978
Has Material Basis InGENO_0000149
Disease Node Iddisease_node_13252
Doid IdDOID_0112002
LabelImmunodeficiency 47