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Dna Ligase Iv Deficiency

Disease ID: disease_node_13311

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DbxrefMIM:606593
SubclassofDOID_628, DOID_0050737
Data SourceDOID
SynonymsLIG4 Syndrome
Doid LabelDNA ligase IV deficiency
Doid DescriptionA combined T cell and B cell immunodeficiency that has_material_basis_in a mutation in the LIG4 gene, a DNA ligase, encoding a protein essential for V(D)J recombination and DNA double-strand break (DSB) repair through nonhomologous end joining (NHEJ). Patients present with immunodeficiency and developmental and growth delay. OMIM mapping confirmed by DO. [SN].
Has PhenotypeHP_0001510
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13311
Doid IdDOID_0060021
LabelDna Ligase Iv Deficiency