AUTOSOMAL-DOMINANT ZONULAR CATARACT WITH SUTURAL OPACITIES LOCALIZED TO CHROMOSOME 17Q11-12

Congenital cataracts constitute a morphologically and genetically hete rogeneous group of diseases that are a major cause of childhood blindn ess. Different loci for hereditary congenital cataracts have been mapp ed to chromosomes 1, 2, 16, and 17q24. We report linkage of a gene cau sing a unique form of autosomal dominant zonular cataracts with Y-sutu ral opacities to chromosome 17q11-12 in a three-generation family exhi biting a maximum lod score of 3.9 at D17S805. Multipoint analysis gave a l-lod confidence interval of 17 cM. This interval is bounded by the markers D17S799 and D17S798, a region that would encompass a number o f candidate genes including that coding for beta A3/A1-crystallin.