特異なapo E分布を呈した症候性低βリポ蛋白質血症の1例

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タイトル別名
  • A Case of Symptomatic Hypobetalipoproteinemia with Unusual Distribution of Apolipoprotein E.

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A case of symptomatic hypobetalipoproteinemia (hypo-βLP) with unusual distribution of apolipoprotein E (apo E) in a 68-year-old male patient with chronic heart failure and liver cirrhosis associated with low triiodothyronine (T3) syndrome is reported. There was nothing in the family history to suggest familial hypo-β LP. In this case, levels of apo B and low-density lipoprotein were very low, and the fraction of β lipoprotein on polyacrylamide-gel disc electrophoresis (PAGE) was only 7%. However, the triglyceride level was normal due to the presence of chylomicron, in spite of hypocholesterolemia and hypophospholipidemia. The mid-band lipoprotein on PAGE showed that Lp (a) lipoprotein concentration was normal (18.3mg/dl). The activities of lecithin cholesterol acyltransferase, hepatic triglyceride lipase and lipoprotein lipase (LPL) were low. The concentrations of apo C-II, apo C-III and apo E were low, while those of apo A-I and apo A-II were normal. The author recently reported that the apo C of high-density lipoprotein (HDL-apo C) was detected in α lipoprotein, but that HDL-apo E was detected in the near α2-globulin region behind α lipoprotein on agarose-gel immunofixation electrophoresis. The author therefore named it α2- apo E, and later found that the fraction percentage of α2-apo E depends on lipolysis and is inversely correlated to the concentration of apo B. It was confirmed that apo C-III and apo E in 22 patients with symptomatic hypo-β LP were distributed for the most part in the HDL and near the α2-globulin regions respectively, and that the percentage of α2-apo E in 10 patients with low T3 syndrome was higher than that in 20 healthy controls. However, in the present case α2-apo E was absent, so that apo E was distributed only in chylomicron and pre-β lipoprotein, while apo C-III was distributed mostly in HDL and, to a lesser extent, in the apo B-containing lipoproteins. It is suspected that the unusual distribution of apo E in this case is due to the decrease of LPL activity.

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