Saito H, Goodnough L T, Soria J, Soria C, Aznar J, España F
N Engl J Med. 1981 Oct 15;305(16):910-4. doi: 10.1056/NEJM198110153051602.
We studied the plasma of 18 patients with a functional deficiency of plasma prekallikrein (Fletcher trait). Samples from 13 subjects contained less than 1 per cent of normal levels of prekallikrein antigen recognized by a specific antiserum (cross-reacting-material negative [CRM-]). In the five other subjects, however, nonfunctional material immunologically indistinguishable from normal prekallikrein was detected by radioimmunoassays at concentrations of 13 to 30 per cent (CRM+ variant). None of the plasma samples contained detectable circulating anticoagulants against prekallikrein. On immunodiffusion against antiserum to kallikrein, each of the five CRM+ samples formed a single precipitin line of complete identity with normal plasma or purified prekallikrein. On immunoelectrophoresis, the precipitin line had the same mobility as that for normal prekallikrein. These studies demonstrate the molecular heterogeneity of human prekallikrein deficiency and show that persons with the CRM+ variant have a nonfunctional form of prekallikrein in their plasma.
我们研究了18例血浆前激肽释放酶功能缺陷（弗莱彻性状）患者的血浆。13名受试者的样本中，被特异性抗血清识别的前激肽释放酶抗原含量低于正常水平的1%（交叉反应物质阴性[CRM-]）。然而，在其他5名受试者中，通过放射免疫测定法检测到与正常前激肽释放酶在免疫学上无法区分的无功能物质，其浓度为13%至30%（CRM+变异型）。所有血浆样本均未检测到针对前激肽释放酶的循环抗凝剂。在用抗激肽释放酶抗血清进行免疫扩散时，5个CRM+样本中的每一个都与正常血浆或纯化的前激肽释放酶形成了一条完全相同的单一沉淀线。在免疫电泳中，沉淀线的迁移率与正常前激肽释放酶相同。这些研究证明了人类前激肽释放酶缺乏的分子异质性，并表明CRM+变异型患者的血浆中存在无功能形式的前激肽释放酶。
