{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T09:03:28Z","timestamp":1791018208993,"version":"4.1.0"},"reference-count":14,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2013,4]]},"abstract":"<jats:sec>\n            <jats:title>Background\u2014<\/jats:title>\n            <jats:p>\n              Loss-of-function mutations in Na\n              <jats:sub>v<\/jats:sub>\n              1.5 cause sodium channelopathies, including Brugada syndrome, dilated cardiomyopathy, and sick sinus syndrome; however, no effective therapy exists. MOG1 increases plasma membrane (PM) expression of Na\n              <jats:sub>v<\/jats:sub>\n              1.5 and sodium current (\n              <jats:italic>I<\/jats:italic>\n              <jats:sub>Na<\/jats:sub>\n              ) density, thus we hypothesize that MOG1 can serve as a therapeutic target for sodium channelopathies.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods and Results\u2014<\/jats:title>\n            <jats:p>\n              Knockdown of\n              <jats:italic>MOG1<\/jats:italic>\n              expression using small interfering RNAs reduced Na\n              <jats:sub>v<\/jats:sub>\n              1.5 PM expression, decreased\n              <jats:italic>I<\/jats:italic>\n              <jats:sub>Na<\/jats:sub>\n              densities by 2-fold in HEK\/Na\n              <jats:sub>v<\/jats:sub>\n              1.5 cells and nearly abolished\n              <jats:italic>I<\/jats:italic>\n              <jats:sub>Na<\/jats:sub>\n              in mouse cardiomyocytes. MOG1 did not affect Na\n              <jats:sub>v<\/jats:sub>\n              1.5 PM turnover.\n              <jats:italic>MOG<\/jats:italic>\n              1 small interfering RNAs caused retention of Na\n              <jats:sub>v<\/jats:sub>\n              1.5 in endoplasmic reticulum, disrupted the distribution of Na\n              <jats:sub>v<\/jats:sub>\n              1.5 into caveolin-3\u2013enriched microdomains, and led to redistribution of Na\n              <jats:sub>v<\/jats:sub>\n              1.5 to noncaveolin-rich domains. MOG1 fully rescued the reduced PM expression and\n              <jats:italic>I<\/jats:italic>\n              <jats:sub>Na<\/jats:sub>\n              densities by Na\n              <jats:sub>v<\/jats:sub>\n              1.5 trafficking\u2013defective mutation D1275N associated with sick sinus syndrome\/dilated cardiomyopathy\/atrial arrhythmias. For Brugada syndrome mutation G1743R, MOG1 restored the impaired PM expression of the mutant protein and restored\n              <jats:italic>I<\/jats:italic>\n              <jats:sub>Na<\/jats:sub>\n              in a heterozygous state (mixture of wild type and mutant Na\n              <jats:sub>v<\/jats:sub>\n              1.5) to a full level of a homozygous wild-type state.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions\u2014<\/jats:title>\n            <jats:p>\n              Use of MOG1 to enhance Na\n              <jats:sub>v<\/jats:sub>\n              1.5 trafficking to PM may be a potential personalized therapeutic approach for some patients with Brugada syndrome, dilated cardiomyopathy, and sick sinus syndrome in the future.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circep.111.000206","type":"journal-article","created":{"date-parts":[[2013,2,19]],"date-time":"2013-02-19T13:41:46Z","timestamp":1361281306000},"page":"392-401","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":57,"title":["MOG1 Rescues Defective Trafficking of Na\n            <sub>v<\/sub>\n            1.5 Mutations in Brugada Syndrome and Sick Sinus Syndrome"],"prefix":"10.1161","volume":"6","author":[{"given":"Susmita","family":"Chakrabarti","sequence":"first","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaofen","family":"Wu","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaogang","family":"Yang","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ling","family":"Wu","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandro L.","family":"Yong","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cuntai","family":"Zhang","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keli","family":"Hu","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing K.","family":"Wang","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiuyun","family":"Chen","sequence":"additional","affiliation":[{"name":"From the Department of Molecular Cardiology, Center for Cardiovascular Genetics, Lerner Research Institute, Cleveland Clinic, Cleveland, OH (S.C., X.W., L.W., S.L.Y., Q.K.W., Q.C.); Department of Molecular Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH (S.C., Q.K.W., Q.C.); Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (X.W., C.Z.); Division of Pharmacology, College of Pharmacy, Ohio..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/32675"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0010985"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M709721200"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0854.2001.21109.x"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCGENETICS.110.959130"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pbiomolbio.2008.10.005"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardiores.2004.01.022"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M406906200"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.78.5.916"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1161\/hh0402.105177"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.118.suppl_18.S_338-c"},{"key":"e_1_3_4_13_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1109370109"},{"key":"e_1_3_4_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.vascn.2004.08.014"},{"key":"e_1_3_4_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.987248"}],"container-title":["Circulation: Arrhythmia and Electrophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCEP.111.000206","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T00:21:41Z","timestamp":1715559701000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCEP.111.000206"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,4]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,4]]}},"alternative-id":["10.1161\/CIRCEP.111.000206"],"URL":"https:\/\/doi.org\/10.1161\/circep.111.000206","relation":{},"ISSN":["1941-3149","1941-3084"],"issn-type":[{"value":"1941-3149","type":"print"},{"value":"1941-3084","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,4]]},"assertion":[{"value":"2012-08-23","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-01-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-02-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}