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- IPEX_syndrome abstract "IPEX (immunodysregulation polyendocrinopathy enteropathy X-linked syndrome) is a rare disease linked to the dysfunction of the transcription factor FOXP3, widely considered to be the master regulator of the regulatory T cell lineage.It leads to the dysfunction of regulatory T-cells and the subsequent autoimmunity. The disorder manifests with autoimmune enteropathy, psoriasiform or eczematous dermatitis, nail dystrophy, autoimmune endocrinopathies (overlapping with those seen in APECED syndrome), and autoimmune skin conditions such as alopecia universalis and bullous pemphigoid.This autoimmunity is called IPEX and it is the attack from the body's own immune system against the body's own tissues and organs. Early age onset of this disease in males causes severe enlargement of the secondary lymphoid organs, insulin dependent diabetes, eczema, food allergies, and infections. There has been limited success in treating the syndrome by bone marrow transplantation.".
- IPEX_syndrome diseasesdb "33417".
- IPEX_syndrome omim "304790".
- IPEX_syndrome wikiPageExternalLink NBK1118.
- IPEX_syndrome wikiPageID "4104309".
- IPEX_syndrome wikiPageRevisionID "591816534".
- IPEX_syndrome diseasesdb "33417".
- IPEX_syndrome genereviewsname "IPEX Syndrome".
- IPEX_syndrome genereviewsnbk "NBK1118".
- IPEX_syndrome hasPhotoCollection IPEX_syndrome.
- IPEX_syndrome name "IPEX syndrome".
- IPEX_syndrome omim "304790".
- IPEX_syndrome subject Category:Autoimmune_diseases.
- IPEX_syndrome subject Category:Diseases_of_immune_dysregulaton.
- IPEX_syndrome subject Category:Rare_diseases.
- IPEX_syndrome subject Category:Transcription_factor_deficiencies.
- IPEX_syndrome type Abstraction100002137.
- IPEX_syndrome type Attribute100024264.
- IPEX_syndrome type Condition113920835.
- IPEX_syndrome type Lack114449405.
- IPEX_syndrome type Need114449126.
- IPEX_syndrome type State100024720.
- IPEX_syndrome type TranscriptionFactorDeficiencies.
- IPEX_syndrome type Disease.
- IPEX_syndrome type AilmentCondition.
- IPEX_syndrome type Situation.
- IPEX_syndrome comment "IPEX (immunodysregulation polyendocrinopathy enteropathy X-linked syndrome) is a rare disease linked to the dysfunction of the transcription factor FOXP3, widely considered to be the master regulator of the regulatory T cell lineage.It leads to the dysfunction of regulatory T-cells and the subsequent autoimmunity.".
- IPEX_syndrome label "IPEX syndrome".
- IPEX_syndrome label "IPEX症候群".
- IPEX_syndrome label "Ipex".
- IPEX_syndrome sameAs Ipex.
- IPEX_syndrome sameAs m.0bj7j6.
- IPEX_syndrome sameAs Q4347134.
- IPEX_syndrome sameAs Q4347134.
- IPEX_syndrome sameAs IPEX_syndrome.
- IPEX_syndrome wasDerivedFrom IPEX_syndrome?oldid=591816534.
- IPEX_syndrome isPrimaryTopicOf IPEX_syndrome.
- IPEX_syndrome name "IPEX syndrome".