Abstract
Analysis of three pedigrees (including one of a Negro family) presented in this report clearly indicates that the hypocalcified type of amelogenesis imperfecta is transmitted as an autosomal dominant trait with uniform gene expression and complete penetrance. Males and females were affected in equal numbers in these kindreds. The findings are consistent with a single-gene hypothesis and lend support to the concept that this type of amelogenesis imperfecta is a distinct genetic entity.
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