A DNA PROBE FOR THE LDL RECEPTOR GENE IS TIGHTLY LINKED TO HYPERCHOLESTEROLEMIA IN A PEDIGREE WITH EARLY CORONARY-DISEASE

  • 1 September 1986
    • journal article
    • research article
    • Vol. 39  (3) , 300-306
Abstract
A large, multigenerational family with dominantly inherited hypercholesterolemia was analyzed for genetic linkage between blood levels of low-density lipoprotin (LDL) cholesterol and the locus for the LDL receptor. A genetic marker was identified by restriction fragment length polymorphism (RFLP) in a cloned segment of the LDL receptor gene. We found no exceptions to segregation of the high-LDL cholesterol phenotype with a unique allele at the LDL receptor locus in this pedigree; tight linkage was indicated by a maximum lod score of 7.52 at .theta. = 0. Knowledge of the LDL receptor genotype will enable investigators to study variability of phenotypic expression in response to environmental influences or to different genetic determinants.