LIG4 syndrome is a hereditary disorder associated with impaired DNA double-strand break repair mechanisms and characterized by microcephaly, unusual facial features, growth and developmental delay, skin anomalies, and pancytopenia, which is associated with combined immunodeficiency (CID). [ Orphanet:99812 ]

Synonyms: ligase 4 syndrome DNA ligase IV deficiency LIG4 syndrome

This is just here as a test because I lose it

Term information

database cross reference
  • NCIT:C122657 (MONDO:equivalentTo)
  • SCTID:724177005 (MONDO:equivalentTo)
  • DOID:0060021 (MONDO:equivalentTo)
  • GARD:15000 (MONDO:GARD)
  • MESH:C564694 (MONDO:equivalentTo)
  • MEDGEN:339855 (MONDO:equivalentTo)
  • UMLS:C1847827 (MONDO:equivalentTo)
  • Orphanet:99812 (OMIM:606593)
  • OMIM:606593 (Orphanet:99812/e)
Subsets

matrix_llm__is_cancer_other, ordo_disorder, gard_rare, mondo_top_grouping_member, matrix_llm__txgnn_other, harrisons_view_immune_system_disorder, matrix_included, nord_rare, clingen, mondo_top_grouping_immune_system_disorder, rare, orphanet_rare, mondo_top_grouping_syndromic_disease, matrix_llm__is_glucose_dysfunction_other, otar, matrix_llm__tag_qualy_lost_other, harrisons_view_disorder_of_development_or_morphogenesis, matrix_llm__medical_specialization_other, mondo_top_grouping_hereditary_disease, matrix_llm__tag_existing_treatment_other, matrix_txgnn_grouping_other, matrix_llm__anatomical_other, matrix_llm__is_pathogen_caused_other, harrisons_view_member, harrisons_view_syndromic_disease, harrisons_view_hereditary_disease, mondo_top_grouping_disorder_of_development_or_morphogenesis

ClinGen label
DNA ligase IV deficiency [ DOID:0060021 Orphanet:99812 ]

curated content resource

https://search.clinicalgenome.org/kb/conditions/MONDO:0011686

exactMatch

https://omim.org/entry/606593

http://www.orpha.net/ORDO/Orphanet_99812

http://linkedlifedata.com/resource/umls/id/C1847827

http://purl.obolibrary.org/obo/NCIT_C122657

http://identifiers.org/mesh/C564694

http://identifiers.org/medgen/339855

http://identifiers.org/snomedct/724177005

http://purl.obolibrary.org/obo/DOID_0060021

excluded subClassOf

http://purl.obolibrary.org/obo/MONDO_0018814

id

MONDO:0011686