Title: immunodysregulation–polyendocrinopathy–enteropathy, X-linked
Long Title: IUPAC Gold Book - immunodysregulation–polyendocrinopathy–enteropathy, X-linked
DOI: 10.1351/goldbook.13283
Status: current

Definition
X-linked syndrome characterized by immunodysregulation, polyendocrinopathy (diabetes mellitus type 1, thyroiditis), hemolytic anemia, thrombocytopenia, dermatitis, and enteropathy, caused by mutations in the gene encoding Foxp3.

Related Terms
- Foxp3: https://goldbook.iupac.org/terms/view/13141
- dermatitis: https://goldbook.iupac.org/terms/view/13089
- diabetes mellitus type 1: https://goldbook.iupac.org/terms/view/13092
- hemolytic anemia: https://goldbook.iupac.org/terms/view/13202
- thyroiditis: https://goldbook.iupac.org/terms/view/13741

Source
- PAC, 2012, 84, 1113. 'IUPAC glossary of terms used in immunotoxicology (IUPAC Recommendations 2012)' on page 1186 (https://doi.org/10.1351/PAC-REC-11-06-03)

Other Outputs
- html: https://goldbook.iupac.org/terms/view/13283/html
- json: https://goldbook.iupac.org/terms/view/13283/json
- xml: https://goldbook.iupac.org/terms/view/13283/xml

Citation: Citation: 'immunodysregulation–polyendocrinopathy–enteropathy, X-linked' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. 10.1351/goldbook.13283
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Accessed: 2026-05-10T19:33:33+00:00