Knot E A, de Jong E, ten Cate J W, Iburg A H, Henny C P, Bruin T, Stibbe J
Blood. 1986 Jan;67(1):93-8.
Purified human radioiodinated antithrombin III (125I-AT III) was used to study its metabolism in six members from three different families with a known hereditary AT III deficiency. Six healthy volunteers served as a control group. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and crossed immunoelectrophoresis (CIE) showed the purified AT III to be homogeneous. Amino acid analysis of the protein revealed a composition identical to a highly purified internal standard. The specific activity was 5.6 U/mg. Analysis of plasma radioactivity data was performed, using a three-compartment model. Neither plasma disappearance half-times nor fractional catabolic rate constants differed significantly between patients and control subjects. The mean absolute catabolic rate in the patient group was significantly lower than that of the control group at 2.57 +/- 0.44 and 4.46 +/- 0.80 mg/kg/day, respectively. In addition, the mean patient alpha 1-phase, flux ratio (k1,2 and k2,1) of the second compartment alpha 2-phase and influx (k3,1) of the third compartment were significantly reduced as compared with control values. It has been tentatively concluded that the observed reduction in the second compartment may be caused by a decrease in endothelial cell surface binding.
纯化的人放射性碘化抗凝血酶III（125I-AT III）用于研究其在来自三个不同家族的六名已知遗传性AT III缺乏症患者中的代谢情况。六名健康志愿者作为对照组。十二烷基硫酸钠-聚丙烯酰胺凝胶电泳（SDS-PAGE）和交叉免疫电泳（CIE）显示纯化的AT III是均一的。该蛋白质的氨基酸分析显示其组成与高度纯化的内标相同。比活性为5.6 U/mg。使用三室模型对血浆放射性数据进行分析。患者和对照受试者之间的血浆消失半衰期和分数分解代谢率常数均无显著差异。患者组的平均绝对分解代谢率分别为2.57±0.44和4.46±0.80 mg/kg/天，显著低于对照组。此外，与对照值相比，患者第二室的平均α1期、通量比（k1,2和k2,1）、第二室α2期和第三室的流入量（k3,1）均显著降低。初步得出结论，观察到的第二室减少可能是由于内皮细胞表面结合减少所致。
