Fiorelli G, Manoussakis C, Sampietro M, Pittalis S, Guglielmino C R, Cappellini M D
Istituto di Scienze Biomediche, Monza, Italy.
Ann Hum Genet. 1989 Jul;53(3):229-36. doi: 10.1111/j.1469-1809.1989.tb01789.x.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is common in Italy and recent biochemical and kinetic studies have demonstrated the presence of polymorphic variants associated with severe or mild enzyme deficiency. We performed a biochemical characterization of G6PD in a large number of Italian G6PD deficient men in an attempt to identify the most reliable biochemical indices for discriminating polymorphic G6PD variants and to define their distribution throughout the country. Three polymorphic G6PD variants, respectively named Mediterranean, Cagliari and Sassari, were identified. The elution profile on DEAE-Sephadex column chromatography, substrate analogue utilization and the Michaelis constant for G6P were the most significant parameters to discriminate polymorphic variants among the variants with activity levels lower than 0.8 IU/g Hb. The distribution of the polymorphic variants, in relation to the origin of the subjects studied, differs throughout the country.
葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症在意大利很常见,最近的生化和动力学研究表明存在与严重或轻度酶缺乏相关的多态性变体。我们对大量意大利G6PD缺乏症男性进行了G6PD的生化特征分析,试图确定区分多态性G6PD变体的最可靠生化指标,并确定它们在全国的分布情况。鉴定出了三种多态性G6PD变体,分别命名为地中海型、卡利亚里型和萨萨里型。在DEAE-葡聚糖凝胶柱色谱上的洗脱曲线、底物类似物的利用情况以及G6P的米氏常数是区分活性水平低于0.8 IU/g Hb的变体中多态性变体的最重要参数。多态性变体的分布与所研究对象的来源有关,在全国范围内有所不同。