Griffith M J, Carraway T, White G C, Dombrose F A
Blood. 1983 Jan;61(1):111-8.
Plasma levels of antithrombin-heparin cofactor, determined by heparin-dependent antithrombin assay, and antithrombin III antigen were measured in 22 members of a large kindred predisposed to venous thrombosis. While 11 members had reduced plasma levels of both antithrombin-heparin cofactor and antithrombin III antigen, the levels of antithrombin-heparin cofactor were always greater than the levels of antithrombin III antigen: 66% (+/- 7%) and 49% (+/- 5%) of normal plasma, respectively. Pooled normal plasma and plasma from one of the affected family members (60% antithrombin-heparin cofactor and 47% antithrombin III antigen) were fractionated by heparin-agarose affinity chromatography. Antithrombin-heparin cofactor, which eluted from heparin-agarose with buffer containing 0.4 M NaCl and did not cross-react with antibody specific for antithrombin III and did not inhibit factor Xa at an appreciable rate in the presence of heparin, was designated heparin cofactor A. Antithrombin-heparin cofactor, which eluted from heparin-agarose with buffer containing 2.0 M NaCl, was functionally and antigenically identified as antithrombin III. The concentrations of heparin cofactor A in normal and patient plasma were similar (4.5 x 10(-7) M), while the concentration of antithrombin III in patient plasma (8.0 x 10(-7) M) was only 50% of normal (1.6 x 10(-6) M). The functional properties of both heparin cofactor A and antithrombin III obtained from patient plasma were normal. From the results of the present study it would appear that the antithrombin-heparin cofactor concentrating measured in patient plasma reflects the combined concentrations of heparin cofactor A and antithrombin III. Since heparin cofactor A does not cross-react with antibody to antithrombin III, the concentration of antithrombin III antigen in patient plasma is thus lower than the concentration of antithrombin-heparin cofactor.
通过肝素依赖抗凝血酶测定法测定了一个易患静脉血栓形成的大家族中22名成员的血浆抗凝血酶-肝素辅因子水平和抗凝血酶III抗原水平。11名成员的血浆抗凝血酶-肝素辅因子和抗凝血酶III抗原水平均降低，但抗凝血酶-肝素辅因子水平始终高于抗凝血酶III抗原水平，分别为正常血浆的66%（±7%）和49%（±5%）。将混合的正常血浆和一名患病家庭成员的血浆（抗凝血酶-肝素辅因子为60%，抗凝血酶III抗原为47%）通过肝素-琼脂糖亲和层析进行分离。从肝素-琼脂糖中用含0.4M NaCl的缓冲液洗脱、不与抗凝血酶III特异性抗体发生交叉反应且在肝素存在下不以可观速率抑制因子Xa的抗凝血酶-肝素辅因子被命名为肝素辅因子A。从肝素-琼脂糖中用含2.0M NaCl的缓冲液洗脱的抗凝血酶-肝素辅因子在功能和抗原性上被鉴定为抗凝血酶III。正常血浆和患者血浆中肝素辅因子A的浓度相似（4.5×10⁻⁷M），而患者血浆中抗凝血酶III的浓度（8.0×10⁻⁷M）仅为正常浓度（1.6×10⁻⁶M）的50%。从患者血浆中获得的肝素辅因子A和抗凝血酶III的功能特性均正常。从本研究结果来看，患者血浆中测得的抗凝血酶-肝素辅因子浓度反映了肝素辅因子A和抗凝血酶III的联合浓度。由于肝素辅因子A不与抗凝血酶III抗体发生交叉反应，因此患者血浆中抗凝血酶III抗原的浓度低于抗凝血酶-肝素辅因子的浓度。
