In vivo metabolism of proapolipoprotein A-I in Tangier disease.

作者: D Bojanovski , R E Gregg , L A Zech , M S Meng , C Bishop

DOI: 10.1172/JCI113266

关键词: CatabolismBiologyApolipoproteins AHypolipoproteinemiaInternal medicineIn vivo metabolismTangier diseaseFamilial disorderIn vivoEndocrinologyApolipoprotein 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

参考文章(22)
H. Bryan Brewer, Rosemary Ronan, Martha Meng, Cheri Bishop, [10] Isolation and characterization of apolipoproteins A-I, A-II, and A-IV Methods in Enzymology. ,vol. 128, pp. 223- 246 ,(1986) , 10.1016/0076-6879(86)28070-2
V I Zannis, A M Lees, R S Lees, J L Breslow, Abnormal apoprotein A-I isoprotein composition in patients with Tangier disease. Journal of Biological Chemistry. ,vol. 257, pp. 4978- 4986 ,(1982) , 10.1016/S0021-9258(18)34621-0
D Bojanovski, R E Gregg, H B Brewer, Tangier disease. In vitro conversion of proapo-A-ITangier to mature APO-A-ITangier. Journal of Biological Chemistry. ,vol. 259, pp. 6049- 6051 ,(1984) , 10.1016/S0021-9258(20)82101-2
C Edelstein, J I Gordon, K Toscas, H F Sims, A W Strauss, A M Scanu, In vitro conversion of proapoprotein A-I to apoprotein A-I. Partial characterization of an extracellular enzyme activity. Journal of Biological Chemistry. ,vol. 258, pp. 11430- 11433 ,(1983) , 10.1016/S0021-9258(17)44242-6
S W Law, H B Brewer, Tangier disease. The complete mRNA sequence encoding for preproapo-A-I. Journal of Biological Chemistry. ,vol. 260, pp. 12810- 12814 ,(1985) , 10.1016/S0021-9258(17)38949-4
A. Lewis Farr, Oliver H. Lowry, Rose J. Randall, Nira J. Rosebrough, Protein Measurement with the Folin Phenol Reagent Journal of Biological Chemistry. ,vol. 193, pp. 265- 275 ,(1951)
H. B. Brewer, R. E. Gregg, S. W. Law, J. M. Hoeg, L. A. Zech, Human apolipoprotein A-I and A-II metabolism Journal of Lipid Research. ,vol. 23, pp. 850- 862 ,(1982) , 10.1007/978-3-642-71702-4_44
D L Sprecher, L Taam, H B Brewer, Two-dimensional electrophoresis of human plasma apolipoproteins. Clinical Chemistry. ,vol. 30, pp. 2084- 2092 ,(1984) , 10.1093/CLINCHEM/30.12.2084
J I Gordon, H F Sims, S R Lentz, C Edelstein, A M Scanu, A W Strauss, Proteolytic processing of human preproapolipoprotein A-I. A proposed defect in the conversion of pro A-I to A-I in Tangier's disease. Journal of Biological Chemistry. ,vol. 258, pp. 4037- 4044 ,(1983) , 10.1016/S0021-9258(18)32773-X
C Edelstein, J I Gordon, C A Vergani, A L Catapano, V Pietrini, A M Scanu, Comparative in vitro study of the pro-apolipoprotein A-I to apolipoprotein A-I converting activity between normal and Tangier plasma. Journal of Clinical Investigation. ,vol. 74, pp. 1098- 1103 ,(1984) , 10.1172/JCI111477