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Iridogoniodysgenesis, dominant type

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Title: Iridogoniodysgenesis, dominant type  
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Subject: Atrichia with papular lesions, PITX2, Tooth and nail syndrome, Limb–mammary syndrome, Enlarged vestibular aqueduct
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Iridogoniodysgenesis, dominant type

Iridogoniodysgenesis, dominant type
Classification and external resources
OMIM 601631
DiseasesDB 34611

Iridogoniodysgenesis, dominant type (type 1, IRID1) refers to a spectrum of diseases characterized by malformations of the irido-corneal angle of the anterior chamber of the eye. Iridogoniodysgenesis is the result of abnormal migration or terminal induction of neural crest cells. These cells lead to formation of most of the anterior segment structures of the eye (corneal stroma & endothelium, iris stroma, trabeculum). [1]

Symptoms: iris hypoplasis, goniodysgenesis, and juvenile glaucoma. Glaucoma phenotype that maps to 6p25 results from mutations in the forkhead transcription factor gene FOXC1

Transmission: autosomal dominant pattern with complete penetrance and variable expressivity.

First reported by Berg (1932). [2]

Treatment: of glaucoma in iridogoniodysgenesis is primarily surgical.

It is listed as a "rare disease" by the Office of Rare Diseases (ORD).[3] This means that Iridogoniodysgenesis, dominant type, or a subtype of Iridogoniodysgenesis, dominant type, affects less than 200,000 people in the US population.


  1. ^ Dureau P.Iridogoniodysgenesis dominant type. Orphanet Encyclopedia. March 2004
  2. ^ Berg F (1932). "Erbliches jugendliches Glaukom". Acta Ophthal. 10: 568–87.  
  3. ^ Iridogoniodysgenesis, dominant type at NIH's Office of Rare Diseases

External links

  • Entrez Gene
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