NOTCH3 p.Arg1231Cys is markedly enriched in South Asians and associated with stroke

Regeneron Genetics Center, Business Operations & Administrative Coordinators, Senior Partnerships & Business Operations, Research Program Management & Strategic Initiatives, Therapeutic Area Genetics, Analytical Genetics and Data Science, Genome Informatics & Data Engineering, Clinical Informatics, Sequencing & Lab Operations, RGC Management & Leadership Team

Research output: Contribution to journalArticlepeer-review

Abstract

The genetic factors of stroke in South Asians are largely unexplored. Exome-wide sequencing and association analysis (ExWAS) in 75 K Pakistanis identified NM_000435.3(NOTCH3):c.3691 C > T, encoding the missense amino acid substitution p.Arg1231Cys, enriched in South Asians (alternate allele frequency = 0.58% compared to 0.019% in Western Europeans), and associated with subcortical hemorrhagic stroke [odds ratio (OR) = 3.39, 95% confidence interval (CI) = [2.26, 5.10], p = 3.87 × 10−9), and all strokes (OR [CI] = 2.30 [1.77, 3.01], p = 7.79 × 10−10). NOTCH3 p.Arg231Cys was strongly associated with white matter hyperintensity on MRI in United Kingdom Biobank (UKB) participants (effect [95% CI] in SD units = 1.1 [0.61, 1.5], p = 3.0 × 10−6). The variant is attributable for approximately 2.0% of hemorrhagic strokes and 1.1% of all strokes in South Asians. These findings highlight the value of diversity in genetic studies and have major implications for genomic medicine and therapeutic development in South Asian populations.

Original languageEnglish
Article number8029
JournalNature Communications
Volume15
Issue number1
DOIs
Publication statusPublished - Dec 2024

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