遗传癌症基因的变异分类受到混合群体基因组代表性不足的影响。

IF 2.1 3区 生物学 Q3 BIOCHEMISTRY & MOLECULAR BIOLOGY
Bianca Caroline Figueiredo Bianco, Aline Cristiane Planello
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引用次数: 0

摘要

基因检测中的变异分类往往以“不确定”的结果告终,即不确定意义的变异(VUS),这仍然是一个主要的临床挑战。在已建立的变异分类标准中,种群等位基因频率(AF)是基本的,但非欧洲群体的代表性不足阻碍了准确的解释。在这项研究中,我们评估了人群特异性房颤对VUS重分类和遗传癌症基因冲突变异的影响。从ClinVar中,我们筛选了巴西队列ABraOM和gnomAD v4.1数据库中存在的487个变体。比较群体分析显示,43%的共有变异表现出显著不同的AFs (q≤0.01),其中113个(23%)表现出较大的效应量(OR≥4),包括39个VUS。其中,20例VUS在巴西队列中AF较高,超过良性阈值(BS1),而在其他人群中仍然罕见。REVEL和CADD等功能预测工具无法将这些变异与全球罕见的VUS区分开来。将巴西特定的AF与ClinGen VCEP规则集成,降低了15%(3/20)的候选VUS,并解决了5个冲突呼叫。这些研究结果表明,在诊断中应常规纳入区域参考数据集,以减少不确定性,避免在不同人群中进行不适当的临床管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Variant classification of hereditary cancer genes is affected by genomic underrepresentation of admixed populations.

Variant classification in genetic testing often culminates in "uncertain" calls, known as variants of uncertain significance (VUS), which remain a major clinical challenge. Among the established criteria for variant classification, population allele frequency (AF) is fundamental, yet under-representation of non-European groups hinders accurate interpretation. In this study, we evaluated the impact of population-specific AF on the reclassification of VUS and conflicting variants in hereditary cancer genes. From ClinVar, we curated 487 variants present in both the Brazilian cohort ABraOM and gnomAD v4.1 databases. Comparative population analysis revealed that 43% of shared variants showed significantly different AFs (q ≤ 0.01), with 113 (23%) exhibiting large effect sizes (OR ≥ 4), including 39 VUS. Among these, 20 VUS had higher AF in the Brazilian cohort and exceeded benignity thresholds (BS1), while remaining rare in other populations. Functional prediction tools such as REVEL and CADD failed to distinguish these variants from globally rare VUS. Integrating Brazilian‑specific AF with ClinGen VCEP rules downgraded 15% (3/20) of candidate VUS and resolved five conflicting calls. These findings argue for routine incorporation of regional reference datasets in diagnostic curation to reduce uncertainty and avoid inappropriate clinical management in diverse populations.

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来源期刊
Molecular Genetics and Genomics
Molecular Genetics and Genomics 生物-生化与分子生物学
CiteScore
5.10
自引率
3.20%
发文量
134
审稿时长
1 months
期刊介绍: Molecular Genetics and Genomics (MGG) publishes peer-reviewed articles covering all areas of genetics and genomics. Any approach to the study of genes and genomes is considered, be it experimental, theoretical or synthetic. MGG publishes research on all organisms that is of broad interest to those working in the fields of genetics, genomics, biology, medicine and biotechnology. The journal investigates a broad range of topics, including these from recent issues: mechanisms for extending longevity in a variety of organisms; screening of yeast metal homeostasis genes involved in mitochondrial functions; molecular mapping of cultivar-specific avirulence genes in the rice blast fungus and more.
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