Boonyuen Usa, Jacob Beatriz Aira C, Chamchoy Kamonwan, Pengsuk Natnicha, Talukam Sirinyatorn, Petcharat Chanya, Adams Emily R, Edwards Thomas, Boonnak Kobporn, Amran Syazwani Itri, Ab Latif Nurriza, Louis Naveen Eugene
Department of Molecular Tropical Medicine and Genetics, Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand.
Princess Srisavangavadhana Faculty of Medicine, Chulabhorn Royal Academy, Bangkok, Thailand.
Trop Med Int Health. 2025 May;30(5):437-457. doi: 10.1111/tmi.14105. Epub 2025 Mar 13.
Accurate diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency is crucial for relapse malaria treatment using 8-aminoquinolines (primaquine and tafenoquine), which can trigger haemolytic anaemia in G6PD-deficient individuals. This is particularly important in regions where the prevalence of G6PD deficiency exceeds 3%-5%, including Southeast Asia and Thailand. While quantitative phenotypic tests can identify women with intermediate activity who may be at risk, they cannot unambiguously identify heterozygous females who require appropriate counselling. This study aimed to develop a genetic test for G6PD deficiency using high-resolution melting curve analysis, which enables zygosity identification of 15 G6PD alleles. In 557 samples collected from four locations in Thailand, the prevalence of G6PD deficiency based on indirect enzyme assay was 6.10%, with 8.08% exhibiting intermediate deficiency. The developed high-resolution melting assays demonstrated excellent performance, achieving 100% sensitivity and specificity in detecting G6PD alleles compared with Sanger sequencing. Genotypic variations were observed across four geographic locations, with the combination of c.1311C>T and c.1365-13T>C being the most common genotype. Compound mutations, notably G6PD Viangchan (c.871G>A, c.1311C>T and c.1365-13T>C), accounted for 15.26% of detected mutations. The high-resolution melting assays also identified the double mutation G6PD Chinese-4 + Canton and G6PD Radlowo, a variant found for the first time in Thailand. Biochemical and structural characterisation revealed that these variants significantly reduced catalytic activity by destabilising protein structure, particularly in the case of the Radlowo mutation. The refinement of these high-resolution melting assays presents a highly accurate and high-throughput platform that can improve patient care by enabling precise diagnosis, supporting genetic counselling and guiding public health efforts to manage G6PD deficiency-especially crucial in malaria-endemic regions where 8-aminoquinoline therapies pose a risk to deficient individuals.
准确诊断葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症对于使用8-氨基喹啉类药物(伯氨喹和他非诺喹)治疗复发性疟疾至关重要,因为这些药物可在G6PD缺乏的个体中引发溶血性贫血。这在G6PD缺乏症患病率超过3%-5%的地区尤为重要,包括东南亚和泰国。虽然定量表型测试可以识别可能有风险的中等活性女性,但它们无法明确识别需要适当咨询的杂合子女性。本研究旨在开发一种使用高分辨率熔解曲线分析的G6PD缺乏症基因检测方法,该方法能够鉴定15种G6PD等位基因的纯合性。在从泰国四个地点收集的557份样本中,基于间接酶测定的G6PD缺乏症患病率为6.10%,8.08%表现为中度缺乏。所开发的高分辨率熔解分析表现出色,与桑格测序相比,在检测G6PD等位基因时实现了100%的灵敏度和特异性。在四个地理位置观察到基因型变异,c.1311C>T和c.1365-13T>C的组合是最常见的基因型。复合突变,特别是G6PD Viangchan(c.871G>A、c.1311C>T和c.1365-13T>C),占检测到的突变的15.26%。高分辨率熔解分析还鉴定出双突变G6PD Chinese-4 + Canton和G6PD Radlowo,这是在泰国首次发现的变体。生化和结构表征表明,这些变体通过破坏蛋白质结构显著降低了催化活性,特别是在Radlowo突变的情况下。这些高分辨率熔解分析的完善提供了一个高度准确和高通量的平台,通过实现精确诊断、支持遗传咨询和指导公共卫生努力来管理G6PD缺乏症,从而改善患者护理——这在疟疾流行地区尤为关键,因为8-氨基喹啉疗法对缺乏症个体构成风险。