Abstract
We describe a new phenotype of hyperlipoproteinemia in two members of a family with a high degree of consanguinity. Both have a history of uncontrolled diabetes mellitus without ketoacidosis, and a family history of coronary artery disease at a relatively early age. A high degree of insulin resistance was found. The abnormal lipoprotein(s) has alpha-lipoprotein mobility on cellulose acetate electrophoresis and has a relative density of less than 1.006 as determined by ultracentrifugation of serum collected after a short fast. The fraction isolated by ultracentrifugation contains about half of the serum cholesterol and triglycerides and most of the phospholipids; the major protein component is albumin. Immunoelectrophoresis showed low concentrations of beta-lipoproteins in both sera, and two abnormal precipitin bands against monospecific antiserum to antilipoprotein A; a third member of the family showed only one abnormal precipitin band against the same antibody. We tentatively propose an abnormal gene(s) as the underlying mechanism. The insulin-resistant diabetes mellitus, probably inherited separately, may aggravate the hyperlipidemia.
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