OBO ID: DOID:0090110
Term Name: immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome Search Ontology:
Synonyms:
  • Autoimmune enteropathy type 1
  • autoimmunity-immunodeficiency syndrome, X-linked
  • diabetes mellitus, congenital insulin-dependent, with fatal secretory diarrhea
  • diarrhea, polyendocrinopathy, fatal infection syndrome, X-linked
  • DMSD
  • IDDM-secretory diarrhea syndrome
  • immunodeficiency, polyendocrinopathy, and enteropathy, X-linked
  • immunodysregulation, polyendocrinopathy, and enteropathy, X-Linked
  • IPEX
  • X-linked autoimmunity-allergic dysregulation syndrome
  • XLAAD
  • XPID
Definition: An autoimmune disease that is characterized by onset in infancy of refractory diarrhea, endocrinopathies, type 1 diabetes mellitus, and dermatitis that has_material_basis_in X-linked recessive mutation in the forkhead box P3 (FOXP3) gene on chromosome Xp11. (2)
References:
Ontology: Human Disease   ( DOID:0090110 )
Relationships
is a type of:
OTHER immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome PAGES
GENES INVOLVED
Human Gene Zebrafish Ortholog OMIM Term OMIM Phenotype ID
FOXP3 Immunodysregulation, polyendocrinopathy, and enteropathy, X-linked 304790
ZEBRAFISH MODELS No data available
PHENOTYPE No data available

CITATIONS: None
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