Lipoprotein Metabolism1 1In preparation of this study, Dr. Eisenberg was supported in part through the Special Foreign Currency Program of the National Library of Medicine, National Institutes of Health, Public Health Service, U. S. Department of Health, Education, and Welfare, Bethesda, Maryland, under an agreement with the Israel Journal of Medical Sciences, Jerusalem, Israel.

Abnormal plasma lipoproteins and lecithin-cholesterol acyltransferase deficiency in alcoholic liver disease.

A double-antibody radioimmunoassay was used to determine the concentration and distribution of apolipoprotein A-I (apoA-I) in the plasma of patients with Tangier disease. Obligate heterozygotes for Tangier disease had apoA-I levels that were 50% or less of controls, even when estimates of high-density lipoprotein cholesterol concentration were normal. Plasma apoA-I levels in Tangier homozygotes were at most 3% of normal. Most of the apoA-I in the plasma of homozygotes sedimented in the ultracentrifuge at density 1.21 g/ml, and no more than 20% was recovered in the plasma lipoprotein fraction. Radioimmunoassay demonstrated no immunochemical differences between Tangier and control apoA-I.

Abnormal concentration and anomalous distribution of apolipoprotein A-I in Tangier disease

A 16-year-old male (S.F.) and his 21-year-old sister (D.H.) from a large family of Italian and Swedish descent had virtually identical lipoprotein pattern and complete absence of LCAT activity. Both had typical corneal opacities and mild anemia with target cells. S.F., but not D.H., presented with proteinuria, which has increased over three years of follow-up. His kidney biopsy revealed lipid deposits in the glomerular basement membrane. Ten relatives in 4 generations had normal LCAT activity and/or lipoprotein pattern. The patients and their relatives had haptoglobin type 2. Factors that might influence the different clinical presentation in our patients (previous renal disease, diet, abnormal lipoproteins), prognosis, and treatment (diet, enzyme replacement, cholestyramine) are discussed.

Report of Two Patients from a Canadian Family of Italian and Swedish Descent

A 16-year-old male (S.F.) and his 21-year-old sister (D.H.)from a large family of Italian and Swedish descent had virtually identical lipoprotein pattern and complete absence of LCAT activity. Both had typical corneal opacities and mild anemia with target cells. S.F., but not D.H., presented with proteinuria, which has increased over three years of follow-up. His kidney biopsy revealed lipid deposits in the glomerular basement membrane. Ten relatives in 4 generations had normal LCAT activity and/or lipoprotein pattern. The patients and their relatives had haptoglobin type 2. Factors that might influence the different clinical presentation in our patients (previous renal disease, diet, abnormal lipoproteins), prognosis, and treatment (diet, enzyme replacement, cholestyramine) are discussed.

Familial LCAT deficiency. Report of two patients from a Canadian family of Italian and Swedish descent.

Three series of experiments have been performed to study plasma lipoprotein metabolism in familial lecithin: cholesterol acyltransferase deficiency. In one series diets that contained little or no long chain triglyceride decreased the concentrations of several of the lipoproteins, particularly including the large unesterified cholesterol- and lecithin-rich lipoproteins in the class of d 1.019–1.063 g/ml. In the second series of experiments post-heparin lipolytic activity was found to increase the concentrations of the same low density lipoproteins in vivo. In the third series of experiments incubation with lecithin: cholesterol acyltransferase in vitro was found to decrease the unesterified cholesterol and lecithin of the large low density lipoproteins and to increase the cholesteryl ester of normal-sized very low, low, and high density lipoproteins. These findings are compatible with the concept that the large low density lipoproteins accumulate in the patients' plasma because chylomicron surface lipids ...

Plasma Lipoprotein Metabolism in Familial Lecithin: Cholesterol Acyltransferase Deficiency

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Plasma Lipoprotein Metabolism in Familial Lecithin: Cholesterol Acyltransferase Deficiency