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DICR occurs through the type 1 ryanodine receptor (RyR1), which physically interacts with the dihydropyridine receptor Cav1.1 subunit in specific machinery formed with additional essential components including \u03b21a, Stac3 adaptor protein, and junctophilins. Exome sequencing has accelerated the discovery of many novel mutations in genes encoding DICR machinery in various skeletal muscle diseases. However, functional validation is time-consuming because it must be performed in a skeletal muscle environment. In this study, we established a platform of the reconstituted DICR in HEK293 cells. The essential components were effectively transduced into HEK293 cells expressing RyR1 using baculovirus vectors, and Ca2+ release was quantitatively measured with R-CEPIA1er, a fluorescent ER Ca2+ indicator, without contaminant of extracellular Ca2+ influx. In these cells, [K+]-dependent Ca2+ release was triggered by chemical depolarization with the aid of inward rectifying potassium channel, indicating a successful reconstitution of DICR. Using the platform, we evaluated several Cav1.1 mutations that are implicated in malignant hyperthermia and myopathy. We also tested several RyR1 inhibitors; whereas dantrolene and Cpd1 inhibited DICR, procaine had no effect. Furthermore, twitch potentiators such as perchlorate and thiocyanate shifted the voltage dependence of DICR to more negative potentials without affecting Ca2+-induced Ca2+ release. These results well reproduced the findings with the muscle fibers and the cultured myotubes. Since the procedure is simple and reproducible, the reconstituted DICR platform will be highly useful for the validation of mutations and drug discovery for skeletal muscle diseases.<\/jats:p>","DOI":"10.1085\/jgp.202213230","type":"journal-article","created":{"date-parts":[[2022,10,4]],"date-time":"2022-10-04T02:20:31Z","timestamp":1664850031000},"update-policy":"https:\/\/doi.org\/10.1085\/jgp.crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["A reconstituted depolarization-induced Ca2+ release platform for validation of skeletal muscle disease mutations and drug discovery"],"prefix":"10.1085","volume":"154","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3845-2980","authenticated-orcid":true,"given":"Takashi","family":"Murayama","sequence":"first","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5005-9698","authenticated-orcid":true,"given":"Nagomi","family":"Kurebayashi","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuro","family":"Numaga-Tomita","sequence":"additional","affiliation":[{"name":"Department of Molecular Pharmacology, Shinshu University School of Medicine, Matsumoto, Japan 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuya","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoru","family":"Okazaki","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyosuke","family":"Yamashiro","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7662-6742","authenticated-orcid":true,"given":"Tsutomu","family":"Nakada","sequence":"additional","affiliation":[{"name":"Department of Molecular Pharmacology, Shinshu University School of Medicine, Matsumoto, Japan 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuichi","family":"Mori","sequence":"additional","affiliation":[{"name":"Institute of Biomaterials and Bioengineering, Tokyo Medical and Dental University, Tokyo, Japan 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryosuke","family":"Ishida","sequence":"additional","affiliation":[{"name":"Institute of Biomaterials and Bioengineering, Tokyo Medical and Dental University, Tokyo, Japan 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Kagechika","sequence":"additional","affiliation":[{"name":"Institute of Biomaterials and Bioengineering, Tokyo Medical and Dental University, Tokyo, Japan 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7515-3824","authenticated-orcid":true,"given":"Mitsuhiko","family":"Yamada","sequence":"additional","affiliation":[{"name":"Department of Molecular Pharmacology, Shinshu University School of Medicine, Matsumoto, Japan 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9515-6574","authenticated-orcid":true,"given":"Takashi","family":"Sakurai","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Juntendo University School of Medicine, Tokyo, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2022,11,1]]},"reference":[{"key":"2022110114322212300_bib1","doi-asserted-by":"publisher","first-page":"645","DOI":"10.1007\/s12079-018-0477-z","article-title":"Role of defective Ca2+ signaling in skeletal muscle weakness: Pharmacological implications","volume":"12","author":"Agrawal","year":"2018","journal-title":"J. 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