Rimon A, Schiffman S, Feinstein D I, Rapaport S I
Blood. 1976 Aug;48(2):165-74.
A relatively potent antiserum against highly purified, unactivated human factor XI antigen was raised in a rabbit. This antiserum, after concentration, neutralized 50% of the factor XI clotting activity of a standard normal plasma at an antiserum dilution of 1/900. The antiserum was used in a neutralization-inhibition assay to study the relation between factor XI clotting activity and factor XI antigen in plasma from ten unrelated patients with homozygous factor XI deficiency and from 12 heterozygous family members of these patients. No evidence of factor XI antigen significantly in excess of factor XI activity was found in either group. All data to date have been consistent with the hypothesis that hereditary factor XI deficiency represents a genetic disorder resulting from the absence of factor XI molecule. Severity of bleeding in factor XI deficiency could not be correlated with the level of factor XI activity or factor XI antigen.
在一只兔子体内制备了一种针对高度纯化的、未激活的人因子XI抗原的相对强效的抗血清。这种抗血清经浓缩后，在抗血清稀释度为1/900时，可中和标准正常血浆中50%的因子XI凝血活性。该抗血清用于中和抑制试验，以研究10名无亲缘关系的纯合子因子XI缺乏症患者及这些患者的12名杂合子家庭成员血浆中因子XI凝血活性与因子XI抗原之间的关系。两组均未发现因子XI抗原显著超过因子XI活性的证据。迄今为止的所有数据均与以下假设一致，即遗传性因子XI缺乏症是一种由于缺乏因子XI分子而导致的遗传性疾病。因子XI缺乏症患者出血的严重程度与因子XI活性水平或因子XI抗原水平无关。
