Plasma lipoproteins in patients with familial plasma lecithin: cholesterol acyltransferase deficiency: apolipoprotein composition of isolated fractions

Seidel, D.

Verhandlungen der Deutschen Gesellschaft für Innere Medizin 78: 1292


ISSN/ISBN: 0070-4067
PMID: 4665520
Accession: 043961263

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Familial lecithin: cholesterol acyltransferase (LCAT) deficiency has recently been described as an inborn error of metabolism. The enzyme acts upon circulating lipoproteins and catalyses the transfer of fatty acid from the beta position of lecithin to the 3-β-Oh group of free cholesterol. Besides characteristic changes of plasma lipid concentrations, deficiency of this enzyme is followed by changes of the physicochemical properties of the plasma lipoprotein fractions. Heterogeneity and abnormality with regard to the apolipoprotein composition are most pronounced in the low density fraction (d 1,063 to 1,21 g/ml). Three different sub-fraction can be isolated from this density class. a) lipoproteins containing only apolipoprotein B; b) lipoproteins containing apolipoprotein B and apolipoprotein C and c) lipoproteins consisting of apolipoprotein C, lipoprotein are very similar to the abnormal lipoprotein properties of the third (c) lipoprotein are very similar to the abnormal lipoprotein (LP-X) characterizing cholestasis. Since liver disease is often associated with low Lcat activity an important metabolic relationship may exist between structure of plasma lipoproteins, Lcat activity and liver function. (Coauthors: Drs. E. Gjone and J. P. Blomhoff, Oslo.