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The skeletal muscle ryanodine receptor, the major intracellular membrane channel through which calcium ions pass to elicit muscle contraction, is central to calcium ion balance and is hypothesized to be a significant factor for age-related decline in muscle function. The nematode Caenorhabditis elegans is a key model system for the study of human aging, and strains were generated with modified C. elegans ryanodine receptors corresponding to human myopathic variants linked with malignant hyperthermia and related conditions. The altered response of these strains to pharmacological agents reflected results of human diagnostic tests for individuals with these pathogenic variants. Involvement of nerve cells in the C. elegans responses may relate to rare medical symptoms concerning the central nervous system that have been associated with ryanodine receptor variants. These single amino acid modifications in C. elegans also conferred a reduction in lifespan and an accelerated decline in muscle integrity with age, supporting the significance of ryanodine receptor function for human aging.<\/jats:p>","DOI":"10.1534\/g3.117.040535","type":"journal-article","created":{"date-parts":[[2017,3,22]],"date-time":"2017-03-22T00:28:21Z","timestamp":1490142501000},"page":"1451-1461","source":"Crossref","is-referenced-by-count":8,"title":["Aging Effects of <i>Caenorhabditis elegans<\/i> Ryanodine Receptor Variants Corresponding to Human Myopathic Mutations"],"prefix":"10.1093","volume":"7","author":[{"given":"Katie Nicoll","family":"Baines","sequence":"first","affiliation":[{"name":"Leeds Institute of Biomedical and Clinical Sciences, St James\u2019s University Hospital, LS9 7TF, United Kingdom"},{"name":"School of Biology, Faculty of Biological Sciences, University of Leeds, LS2 9JT, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9lia","family":"Ferreira","sequence":"additional","affiliation":[{"name":"School of Biology, Faculty of Biological Sciences, University of Leeds, LS2 9JT, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip M","family":"Hopkins","sequence":"additional","affiliation":[{"name":"Leeds Institute of Biomedical and Clinical Sciences, St James\u2019s University Hospital, LS9 7TF, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie-Anne","family":"Shaw","sequence":"additional","affiliation":[{"name":"Leeds Institute of Biomedical and Clinical Sciences, St James\u2019s University Hospital, LS9 7TF, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian A","family":"Hope","sequence":"additional","affiliation":[{"name":"School of Biology, Faculty of Biological Sciences, University of Leeds, LS2 9JT, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2017,5,1]]},"reference":[{"issue":"6","key":"2021042013193741000_bib1","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1007\/s00438-003-0892-5","article-title":"Genetic analysis of ryanodine receptor function in Caenorhabditis elegans based on unc-68 revertants.","volume":"269","author":"Adachi","year":"2003","journal-title":"Mol. 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