
doi: 10.2169/naika.76.123
pmid: 3572150
本邦第5家系目の家族性レシチンコレステロールアシルトランスフェラーゼ欠損症患者の血中には,中間リポ蛋白(IDL)が存在していた, IDLを放射性トリオレイン(14C-triolein)でラベルし,これを基質として肝性リパーゼを作用させた時,正常人の食後血清から得たIDLを同様な方法で調製した基質に比較して,中性脂肪の水解の低下がみられた.この反応系に正常人血清を添加した時,水解の亢進がみられたが,同時に5, 5′-Dithiobis-(2-nitrobenzoic acid) (DTNB)を加えた時には水解の亢進はみられなかつた.以上のことより本症例におけるIDLの出現は, LCAT欠損によるIDL粒子の構成成分の変化,特にフリーコレステロールの増加がその-因となつていることが推測された.
Adult, Lecithin Cholesterol Acyltransferase Deficiency, Lipoproteins, IDL, Hydrolysis, Lipoproteins, Homozygote, Humans, Female, Hypolipoproteinemias
Adult, Lecithin Cholesterol Acyltransferase Deficiency, Lipoproteins, IDL, Hydrolysis, Lipoproteins, Homozygote, Humans, Female, Hypolipoproteinemias
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