Chassanidis Christos, Boutou Effrossyni, Voskaridou Ersi, Balassopoulou Angeliki
Molecular Genetics Laboratory, Thalassaemia and Hemoglobinopathies Center, Laiko General Hospital, Athens, Greece.
Thalassaemia and Hemoglobinopathies Center, Laiko General Hospital, Athens, Greece.
PLoS One. 2016 Jun 28;11(6):e0157393. doi: 10.1371/journal.pone.0157393. eCollection 2016.
Beta-thalassaemia is one of the most common autosomal recessive disorders worldwide. The disease's high incidence, which is observed in the broader Mediterranean area has led to the establishment of molecular diagnostics' assays to prevent affected births. Therefore, the development of a reliable, cost-effective and rapid scanning method for β globin gene point mutations, easily adapted to a routine laboratory, is absolutely essential. Here, we describe, for the first time, the development of a High-Resolution Melting Analysis (HRMA) approach, suitable for scanning the particularly heterogeneous beta globin gene mutations present in the Greek population, and thus adaptable to the Mediterranean and other areas where these mutations have been identified. Within this context, β globin gene regions containing mutations frequently identified in the Greek population were divided in ten overlapping amplicons. Our reactions' setup allowed for the simultaneous amplification of multiple primer sets and partial multiplexing, thereby resulting in significant reduction of the experimental time. DNA samples from β-thalassaemia patients/carriers with defined genotypes were tested. Distinct genotypes displayed distinguishable melting curves, enabling accurate detection of mutations. The described HRMA can be adapted to a high-throughput level. It represents a rapid, simple, cost-effective, reliable, highly feasible and sensitive method for β-thalassaemia gene scanning.
β地中海贫血是全球最常见的常染色体隐性疾病之一。在地中海广大地区观察到的该疾病的高发病率促使建立了分子诊断检测方法以预防患病婴儿出生。因此,开发一种可靠、经济高效且快速的扫描β珠蛋白基因点突变的方法,使其易于应用于常规实验室,绝对至关重要。在此,我们首次描述了一种高分辨率熔解分析(HRMA)方法的开发,该方法适用于扫描希腊人群中存在的特别异质的β珠蛋白基因突变,因此适用于已发现这些突变的地中海及其他地区。在此背景下,将希腊人群中频繁发现的含突变的β珠蛋白基因区域划分为十个重叠扩增子。我们反应体系的设置允许同时扩增多个引物组并进行部分多重化,从而显著减少了实验时间。对具有明确基因型的β地中海贫血患者/携带者的DNA样本进行了检测。不同的基因型显示出可区分的熔解曲线,能够准确检测突变。所描述的HRMA可适应高通量水平。它代表了一种用于β地中海贫血基因扫描的快速、简单、经济高效、可靠、高度可行且灵敏的方法。