Impero J E, Harrison G M, Nelson T E
Pediatr Res. 1981 Jun;15(6):940-4. doi: 10.1203/00006450-198106000-00012.
An oyster gill ciliostatic factor material has been isolated from the saliva of patients with cystic fibrosis (CF) by utilizing its ability to bind to alpha-amylase. It was quantitatively assayed by its ability to reversibly inhibit rabbit muscle glycogen debranching enzyme. The specificity of this CF factor material was investigated by comparing activities from the saliva of CF homozygotes (patients) varying in age, sex, and the severity of the disease; CF obligate heterozygotes (carriers); normal control subjects who had no family history of CF; non-CF asthmatic and allergic bronchitis patients; non-CF immunologically deficient patients with chronic respiratory problems; non-CF juvenile diabetic patients; non-CF pancreatic insufficiency patients; non-CF patients with obstructive liver cirrhosis; and non-CF patients with ectodermal dysplasia. The results show that the CF factor material isolated from CF saliva is specific to subjects with cystic fibrosis and is not associated with similar non-CE chronic disease states, nor is it produced as a result of an organ pathology associated with CF. There was no correlation between the amount of factor present in an individual CF homozygote sample and the severity of the disease. In the case of both the CF homozygote and heterozygote samples, there was also no correlation in either age or sex and the amount of factor present. The degree of inhibition produced by CF homozygotes compared to CF heterozygotes is characteristic of the autosomal recessive mode of inheritance of CF. This finding appears to associate the isolated CF factor material with the affected CF gene and suggests that the factor material is related in some way to the genetic lesion in CF.
通过利用其与α-淀粉酶结合的能力，从囊性纤维化（CF）患者的唾液中分离出一种牡蛎鳃纤毛抑制因子物质。通过其可逆抑制兔肌肉糖原脱支酶的能力对其进行定量测定。通过比较不同年龄、性别和疾病严重程度的CF纯合子（患者）、CF obligate杂合子（携带者）、无CF家族史的正常对照受试者、非CF哮喘和过敏性支气管炎患者、患有慢性呼吸问题的非CF免疫缺陷患者、非CF青少年糖尿病患者、非CF胰腺功能不全患者、非CF阻塞性肝硬化患者以及非CF外胚层发育不良患者的唾液活性，研究了这种CF因子物质的特异性。结果表明，从CF唾液中分离出的CF因子物质对囊性纤维化患者具有特异性，与类似的非CF慢性疾病状态无关，也不是由与CF相关的器官病理学产生的。个体CF纯合子样本中存在的因子量与疾病严重程度之间没有相关性。在CF纯合子和杂合子样本中，年龄或性别与存在的因子量之间也没有相关性。与CF杂合子相比，CF纯合子产生的抑制程度是CF常染色体隐性遗传模式的特征。这一发现似乎将分离出的CF因子物质与受影响的CF基因联系起来，并表明该因子物质在某种程度上与CF中的遗传损伤有关。
