作者: D Bojanovski , R E Gregg , L A Zech , M S Meng , C Bishop
DOI: 10.1172/JCI113266
关键词: Catabolism 、 Biology 、 Apolipoproteins A 、 Hypolipoproteinemia 、 Internal medicine 、 In vivo metabolism 、 Tangier disease 、 Familial disorder 、 In vivo 、 Endocrinology 、 Apolipoprotein B
摘要: Tangier disease is a rare familial disorder characterized by extremely low levels of apolipoprotein A-I (apoA-I) and high density lipoproteins (HDL). In normal subjects, proapoA-I secreted into plasma converted to mature apoA-I the cleavage amino-terminal six amino acids with major isoprotein in being apoA-I. contrast, there marked relative increase as compared ProapoA-I were isolated from radio-labeled, autologous isoproteins injected subjects. The vivo catabolism conversion subjects quantitated. A comparison rate revealed significantly faster both when fractional was 3.9 d-1 3.6 results indicate that (a) enters pro (b) have apoA-I, (c) catabolized at same (d) catabolize much greater than do Therefore, due decrease resulting rapid pro- not defective