EXTENSIVE RESTRICTION SITE POLYMORPHISM AT THE HUMAN PHENYLALANINE-HYDROXYLASE LOCUS AND APPLICATION IN PRENATAL-DIAGNOSIS OF PHENYLKETONURIA

  • 1 January 1985
    • journal article
    • research article
    • Vol. 37  (4) , 619-634
Abstract
A total of 10 restriction site polymorphisms were identified at the human phenylalanine hydroxylase locus using a full-length human phenylalanine hydroxylase c[complementary]DNA clone as a hybridization probe to analyze human genomic DNA. These polymorphic patterns segregate in a Mendelian fashion and concordantly with the disease state in various PKU [phenylketonuria] kindreds. The frequencies of the restriction site polymorphisms at the human phenylalanine hydroxylase locus among Caucasians are such that the observed heterozygosity in the population is 87.5%. Most families with a history of classical PKU can take advantage of the genetic analysis for prenatal diagnosis and carrier detection of the hereditary disorder.