Isshiki Mariko, Griffen Anthony J, Meissner Paul, Spencer Paulette, Cabana Michael D, Klugman Susan D, Colón Mirtha, Maksumova Zoya, Suglia Shakira, Isasi Carmen R, Greally John M, Raj Srilakshmi M
Departments of Genetics, Albert Einstein College of Medicine, Bronx, New York, United States of America.
Department of Biological Sciences, Graduate School of Science, The University of Tokyo, Tokyo, Japan.
PLoS Genet. 2025 Jun 24;21(6):e1011755. doi: 10.1371/journal.pgen.1011755. eCollection 2025 Jun.
The detection of founder pathogenic variants, those observed in high frequency only in a group of individuals with increased inter-relatedness, can help improve delivery of health care for that community. We identified 16 groups with shared ancestry, based on genomic segments that are shared through identity by descent (IBD), in New York City using the genomic data of 25,366 residents from the All Of Us Research Program and the Mount Sinai BioMe biobank. From these groups we defined 7 as founder populations, mostly communities currently under-represented in medical genomics research, such as Puerto Rican and Garifuna. The enrichment analysis of ClinVar pathogenic or likely pathogenic (P/LP) variants in each group identified 201 of these damaging variants across the seven founder populations. We confirmed disease-causing variants previously reported to occur at increased frequencies in Ashkenazi Jewish and Puerto Rican genetic ancestry groups, but most of the damaging variants identified have not been previously associated with any such founder populations, and most of these founder populations have not been described to have increased prevalence of the associated rare disease. Twenty-two of 47 variants meeting Tier 2 prenatal screening criteria (1/100 carrier frequency within these founder groups) have never previously been reported. We show how population structure studies can provide insights into rare diseases disproportionately affecting under-represented founder populations, delivering a health care benefit but also a potential source of stigmatization of these communities, who should be part of the decision-making about implementation into health care delivery.
对奠基者致病变异(即在一组具有较高亲缘关系的个体中仅以高频出现的变异)的检测,有助于改善该群体的医疗保健服务。我们利用来自“我们所有人研究计划”的25366名纽约市居民以及西奈山生物样本库的基因组数据,根据通过血缘同一性(IBD)共享的基因组片段,在纽约市识别出了16个有共同祖先的群体。从这些群体中,我们定义了7个为奠基者群体,其中大多数是目前在医学基因组学研究中代表性不足的群体,如波多黎各人和加里富纳人。对每个群体中ClinVar致病性或可能致病性(P/LP)变异的富集分析,在这7个奠基者群体中识别出了201个此类有害变异。我们证实了先前报道的在阿什肯纳兹犹太人和波多黎各遗传祖先群体中频率增加的致病变异,但所识别出的大多数有害变异此前并未与任何此类奠基者群体相关联,而且这些奠基者群体中的大多数也未被描述为相关罕见病的患病率增加。47个符合二级产前筛查标准(在这些奠基者群体中的携带频率为1/100)的变异中有22个此前从未被报道过。我们展示了群体结构研究如何能够深入了解对代表性不足的奠基者群体影响尤甚的罕见病,这既能带来医疗保健益处,但也可能给这些群体带来污名化的潜在根源,而这些群体应参与到关于将其纳入医疗保健服务实施的决策过程中。