Poster Presentation The 46th Lorne Conference on Protein Structure and Function 2021

Characterization of the effect of disease-causing genetic variants using protein 3D structural alterations (#418)

Sergio Ruiz-Carmona 1 2 , David B Ascher 1 3 4 , Michael Inouye 1 2 5 6 7
  1. Baker Heart and Diabetes Institute, Melbourne, VIC, Australia
  2. Cambridge Baker Systems Genomics Initiative, Melbourne, Cambridge
  3. Department of Biochemistry and Molecular Biology, University of Melbourne, Melbourne, Victoria, Australia
  4. Department of Biochemistry, University of Cambridge, Cambridge, UK
  5. Department of Public Health and Primary Care, University of Cambridge, Cambridge, UK
  6. The Alan Turing Institute, London, UK
  7. Department of Clinical Pathology, The University of Melbourne, Parkville, Victoria, Australia

During the last decade, novel technological developments in high-throughput DNA sequencing have brought in new information about benign and disease-associated genetic mutations. Over the past years, different studies have looked at the functional impact of missense variants, single nucleotide mutations that cause an amino acid substitution in a protein-coding region of the genome, based on conservation through evolution, sequence or structural information, however, there is still very limited understanding about their outcome.

Studying the structural and functional effects of missense variants is still a challenge, but thanks to the development of structural biology methods it represents a rising research area.

We have developed an automatic tool that allows us to characterise and annotate missense variants considering all public 3D protein structure information. Our tool searches for available data and builds a molecular model of the mutated structure to calculate multiple features such as increase or decrease of interactions or changes in solvent accessible surface area, among others.

We have validated the pipeline with data from the literature and applied it on larger datasets from HGMD and ClinVar, where we have identified some examples of, to date, unknown variant effect that might be explained with structural changes.

We have also identified crucial structural features that, when altered, can help in the explanation for disease onset.

Studying the impact of disease-causing variants and identifying new examples may give us not only a better understanding of the underlying molecular mechanisms of different disorders, but also higher chances of finding better treatments as well as helping to overcome drug resistance or selectivity in cancer, diabetes or other metabolic disorders.

  1. Sumaiya I, et al. Insights into Protein Structural, Physicochemical, and Functional Consequences of Missense Variants in 1,330 Disease-associated Human Genes. bioRxiv. 2019; doi: 10.1101/69325.
  2. Zhou Y, et al. Computational Methods for the Pharmacogenetic Interpretation of Next Generation Sequencing Data. Front. Pharmacol. 2018, 9:1437.
  3. Bhattacharya R, Rose PW, Burley SK, Prlić A. Impact of genetic variation on three dimensional structure and function of proteins. PLoS One. 2017; 12: e0171355.
  4. Gao M, et al. Insights into Disease-Associated Mutations in the Human Proteome through Protein Structural Analysis. Structure. 2015, Jul 7;23(7):1362-9.
  5. Hicks M, et al. Functional characterization of 3D protein structures informed by human genetic diversity. PNAS. 2019, 116 (18) 8960-8965.
  6. Sumaiya Iqbal, et al. Comprehensive characterization of amino acid positions in protein structures reveals molecular effect of missense variants. Proceedings of the National Academy of Sciences Nov 2020, 117 (45) 28201-28211; DOI: 10.1073/pnas.2002660117