Evidence for Deficiency of High Density Lipoprotein Lecithin: Cholesterol Acyltransferase Activity (α-LCAT) in Fish Eye Disease
| Autor: | Leif Holmquist, Lars A. Carlson |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
medicine.medical_specialty
Very low-density lipoprotein food.ingredient Sterol O-acyltransferase Alpha (ethology) Biology Lecithin chemistry.chemical_compound Corneal Opacity High-density lipoprotein food Lecithin Cholesterol Acyltransferase Deficiency Internal medicine Internal Medicine medicine Humans Fish-Eye Disease Aged Reverse cholesterol transport Hypolipoproteinemias medicine.disease Lipids Cholesterol Endocrinology chemistry Electrophoresis Polyacrylamide Gel lipids (amino acids peptides and proteins) Lipoproteins HDL Lipoprotein |
| Zdroj: | Acta Medica Scandinavica. 218:189-196 |
| ISSN: | 0001-6101 |
| DOI: | 10.1111/j.0954-6820.1985.tb08846.x |
| Popis: | In a rare familial condition, fish eye disease, there is a low relative content of cholesteryl esters in the plasma high density lipoproteins (HDL) but a normal content of these lipids in the very low (VLDL) and low (LDL) density lipoproteins. Lecithin: cholesterol acyltransferase (LCAT) is the enzyme which mediates the esterification of free cholesterol in the plasma lipoproteins. In the present investigation, isolated HDL from our two fish eye disease patients were found to be excellent substrates during in vitro incubations with normal LCAT as present in lipoprotein depleted plasma from control subjects. Almost all free cholesterol of these HDL fractions became esterified and concomitantly the abnormally small fish eye disease HDL particles increased to a size in the range of that of normal HDL particles. Lipoprotein depleted plasma from fish eye disease, however, lacked the property of normal plasma to esterify the free cholesterol of HDL isolated from plasma of fish eye disease patients or control subjects. These results have led to the formulation of a new concept implying that two different LCAT activities exist in normal plasma. One of these activities, denoted alpha-LCAT, is specific for HDL (alpha-lipoproteins) and the other, beta-LCAT, is specific for VLDL-LDL (pre beta- and beta-lipoproteins). Fish eye disease according to this notion is classified as an alpha-LCAT deficiency in contrast to the classical LCAT deficiency which probably lacks both alpha- and beta-LCAT activities. |
| Databáze: | OpenAIRE |
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