FAMILIAL GLAUCOMA IRIDOGONIODYSPLASIA MAPS TO A 6P25 REGION IMPLICATED IN PRIMARY CONGENITAL GLAUCOMA AND IRIDOGONIODYSGENESIS ANOMALY

Familial glaucoma iridogoniodysplasia (FGI) is a form. of open-angle g laucoma in which developmental anomalies of the iris and irido-corneal angle are associated with a juvenile-onset glaucoma transmitted as an autosomal dominant trait. A single large family with this disorder wa s examined for genetic linkage to microsatellite markers. A peak LOD s core of 11.63 at a recombination fraction of 0 was obtained with marke r D6S967 mapping to chromosome 6p25. Haplotype analysis places the dis ease gene in a 6.4-cM interval between the markers D6S1713 and D6S1600 . Two novel clinical appearances extend the phenotypic range and provi de evidence of variable expressivity. The chromosome Gp25 region is no w implicated in FGI, primary congenital glaucoma, and iridogoniodysgen esis anomaly. This may indicate the presence of a common causative gen e or, alternatively, a cluster of genes involved in eye development/fu nction.