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Tier 3 — Observational / field trialPeer-reviewed

Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology

Wouter van Rheenen, Rick A. A. van der Spek, Mark K. Bakker, Joke J.F.A. van Vugt, Paul J. Hop, Ramona A. J. Zwamborn, Niek de Klein, Harm-Jan Westra, Olivier B. Bakker, Patrick Deelen, Gemma Shireby, Eilís Hannon, Matthieu Moisse, Denis Baird, Restuadi Restuadi, Egor Dolzhenko, Annelot M. Dekker, Klara Gawor, Henk‐Jan Westeneng, Gijs H.P. Tazelaar, Kristel R. van Eijk, Maarten Kooyman, Ross P. Byrne, Mark A. Doherty, Mark Heverin, Ahmad Al Khleifat, Alfredo Iacoangeli, Aleksey Shatunov, Nicola Ticozzi, Johnathan Cooper‐Knock, Bradley Smith, Marta Gromicho, Siddharthan Chandran, Suvankar Pal, Karen Morrison, Pamela J. Shaw, John Hardy, Richard W. Orrell, Michael Sendtner, Thomas Meyer, Nazlı Başak, Anneke J. van der Kooi, Antonia Ratti, Isabella Fogh, Cinzia Gellera, Giuseppe Lauria, Stefania Corti, Cristina Cereda, Daisy Sproviero, Sandra D’Alfonso, Gianni Sorarú, Gabriele Siciliano, Massimiliano Filosto, Alessandro Padovani, Adriano Chiò, Andrea Calvo, Cristina Moglia, Maura Brunetti, Antonio Canosa, Maurizio Grassano, Ettore Beghi, Elisabetta Pupillo, Giancarlo Logroscino, Beatrice Nefussy, Alma Osmanovic, Angelica Nordin, Yossef Lerner, Michal Zabari, Marc Gotkine, Robert H. Baloh, Shaughn Bell, Patrick Vourc’h, Philippe Corcia, Philippe Couratier, Stéphanie Millecamps, Vincent Meininger, François Salachas, Jesus S. Mora Pardina, Abdelilah Assialioui, Ricardo Rojas-García, Patrick A. Dion, Jay P. Ross, Albert C. Ludolph, Jochen H. Weishaupt, Dávid Brenner, Axel Freischmidt, Gilbert Bensimon, Alexis Brice, Alexandra Durr, C. Payán, Safa Saker-Delye, Nicholas Wood, Simon Topp, Rosa Rademakers, Lukas Tittmann, Wolfgang Lieb, André Franke, Stephan Ripke, Alice Braun, Julia Kraft

Nature Genetics · 2021

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Summary

This large cross-ancestry genetic study identified 15 novel and established risk loci for amyotrophic lateral sclerosis through integrated GWAS, whole-genome sequencing, and cortex eQTL analysis. The findings reveal locus-specific genetic architectures involving rare variants, tandem repeats, and regulatory mechanisms, with evidence supporting a causal role for elevated cholesterol levels and highlighting perturbations in vesicle-mediated transport and autophagy as central disease mechanisms.

UK applicability

This fundamental genetic discovery has potential implications for UK-based ALS research and future therapeutic development, though the study itself does not address UK-specific environmental or clinical factors. UK clinicians and researchers may benefit from these validated genetic risk markers in patient stratification and precision medicine approaches.

Key measures

Genetic risk loci identified; association signals in rare variants and short tandem repeats; regulatory effects via eQTL; regional brain and cell-type enrichment patterns; Mendelian randomisation estimates for cholesterol causality

Outcomes reported

The study identified 15 genetic risk loci for amyotrophic lateral sclerosis through genome-wide association analysis in 29,612 patients and 122,656 controls, combined with whole-genome sequencing and expression analysis. Mendelian randomisation indicated a causal role for high cholesterol levels as an environmental risk factor.

Theme
Nutrition & health
Subject
Dietary fats & fatty acids
Study type
Research
Study design
Genome-wide association study (GWAS) with whole-genome sequencing and expression quantitative trait locus (eQTL) analysis
Source type
Peer-reviewed study
Status
Published
Geography
International
System type
Human clinical
DOI
10.1038/s41588-021-00973-1
Catalogue ID
SNmohdwf5u-aeq6uu

Topic tags

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