Table 2

Ocular phenotypes associated with gene mutations linked to anophthalmia/microphthalmia.

Gene
Locus (Inheritance)
Major (and selected less common) Human Ocular Phenotype(s)
OMIM [54]

SOX2
3q26.3-q27 (AD)
Anophthalmia/microphthalmia
184429
PAX6
11p13 (AD)
Aniridia, (Peters anomaly, autosomal dominant keratopathy, foveal hypoplasia, optic nerve malformations, anophthalmia)
607108
OTX2
14q22 (AD)
Anophthalmia/microphthalmia, (retinal dysplasia, optic nerve malformations)
600037
RAX
18q21.3 (AR)
Anophthalmia/microphthalmia
601881
CHX10
14q24.3 (AR)
Microphthalmia
142993
FOXE3
1p32
Anterior segment dysgenesis, congenital primary aphakia
601094
CRYBA4
22q11.2-q13.1 (AD)
Autosomal dominant cataract, (microphthalmia)
123631

Verma and FitzPatrick Orphanet Journal of Rare Diseases 2007 2:47   doi:10.1186/1750-1172-2-47