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In this family different phenotypic manifestations of these diseases were detected. Three unrelated patients were heterozygous for a deletion of the COL1A1 gene. Two of them suffered congenital cataract and ectopia lentis; another patient presented foveal colobomas. In both affected family members there was a variable degree of involvement of extraocular tissues.

The relationship between the different phenotypic manifestations of this gene carrier in the same and in two generations, the role of the COL1A1 gene in lens degeneration, and the pathogenic mechanism of the mutation are discussed.

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