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K\n                    <jats:sub>ATP<\/jats:sub>\n                    channels are found throughout the cardiovascular system and skeletal muscle and couple cellular metabolism to excitability. AIMS individuals show fatigability, muscle spasms, and cardiac dysfunction. We found reduced exercise performance in mouse models of AIMS harboring premature stop codons in\n                    <jats:italic>ABCC9<\/jats:italic>\n                    . Given the roles of K\n                    <jats:sub>ATP<\/jats:sub>\n                    channels in all muscles, we sought to determine how myopathy arises using tissue\u2010selective suppression of K\n                    <jats:sub>ATP<\/jats:sub>\n                    and found that LoF in skeletal muscle, specifically, underlies myopathy. In isolated muscle, SUR2 LoF results in abnormal generation of unstimulated forces, potentially explaining painful spasms in AIMS. We sought to determine whether excessive Ca\n                    <jats:sup>2+<\/jats:sup>\n                    influx through Ca\n                    <jats:sub>V<\/jats:sub>\n                    1.1 channels was responsible for myopathology but found that the Ca\n                    <jats:sup>2+<\/jats:sup>\n                    channel blocker verapamil unexpectedly resulted in premature death of AIMS mice and that rendering Ca\n                    <jats:sub>V<\/jats:sub>\n                    1.1 channels nonpermeable by mutation failed to reverse pathology; results which caution against the use of calcium channel blockers in AIMS.\n                  <\/jats:p>","DOI":"10.15252\/emmm.202216883","type":"journal-article","created":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T10:00:00Z","timestamp":1683540000000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Skeletal muscle delimited myopathy and verapamil toxicity in SUR2 mutant mouse models of AIMS"],"prefix":"10.1038","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0859-3854","authenticated-orcid":false,"given":"Conor","family":"McClenaghan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya A","family":"Mukadam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9780-8126","authenticated-orcid":false,"given":"Jacob","family":"Roeglin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C","family":"Tryon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5196-4024","authenticated-orcid":false,"given":"Manfred","family":"Grabner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8075-8812","authenticated-orcid":false,"given":"Anamika","family":"Dayal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gretchen A","family":"Meyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4929-2134","authenticated-orcid":false,"given":"Colin G","family":"Nichols","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,5,8]]},"reference":[{"key":"BFEMMM202216883_CR1","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1136\/jmedgenet-2013-102085","volume":"51","author":"M Albaqumi","year":"2014","unstructured":"Albaqumi M, Alhabib FA, Shamseldin HE, Mohammed F, Alkuraya FS (2014) A syndrome of congenital hyperinsulinism and rhabdomyolysis is caused by KCNJ11 mutation. 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