STUDIES OF ALPHA-1-ANTITRYPSIN PHENOTYPES AND METABOLISM

蛋白酶抑制剂（Pi）表型已使用 聚丙烯酰胺凝胶等电聚焦技术 正常受试者和全身性类风湿性关节炎患者 红斑狼疮、干燥综合征和肝细胞 carcinoma. 80名南非黑人患者 肝细胞癌，异常（非- MM）表型占8.7%。 在103名年龄、性别和部落匹配的 对照组相应发生率为12.6%。 没有一 患者或对照具有PiZZ表型。 5%的患者 对照组中有1.9%为Z基因杂合子携带者。 没有肝细胞癌患者的血清低于正常值 α-1-抗胰蛋白酶的浓度，通过火箭评估 免疫电泳 四个杂合子的病人 Z表型没有纤维板层癌。 这些 研究结果表明，α-1-抗胰蛋白酶缺乏并不起作用， 南部非洲肝细胞癌的病因学作用 黑人

Protease inhibitor (Pi) phenotypes have been determined using isoelectric focusing on polyacrylamide gel in populations of normal subjects and patients with rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome and hepatocellular carcinoma. Of 80 unselected southern African Black patients with hepatocellular carcinoma, the incidence of aberrant (non- MM) phenotypes was 8.7%. In 103 age, sex and tribally-matched control subjects the corresponding incidence was 12.6%. None of the patients or controls had the PiZZ phenotype. 5% of patients and 1.9% of controls were heterozygous carriers of the Z gene. No patient with hepatocellular carcinoma had a subnormal serum concentration of alpha-1-antitrypsin, as assessed by rocket immuno-electrophoresis. The four patients with the heterozygous Z phenotypes did not have fibrolamellar carcinomas. These findings suggest that alpha-1-antitrypsin deficiency does not play an etiologic role in hepatocellular carcinoma in southern African Blacks.