{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T15:19:38Z","timestamp":1787757578703,"version":"build-2784847793"},"reference-count":62,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T00:00:00Z","timestamp":1782950400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001674","name":"Leducq Foundation","doi-asserted-by":"publisher","award":["18CVD05"],"award-info":[{"award-number":["18CVD05"]}],"id":[{"id":"10.13039\/501100001674","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"NHLBI","doi-asserted-by":"publisher","award":["R35 HL144980"],"award-info":[{"award-number":["R35 HL144980"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"name":"TANGO2 Research Foundation"},{"DOI":"10.13039\/501100012264","name":"NIH","doi-asserted-by":"publisher","award":["R01HL071158"],"award-info":[{"award-number":["R01HL071158"]}],"id":[{"id":"10.13039\/501100012264","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,8,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>TANGO2 deficiency disorder (TDD), caused by biallelic pathogenic variants in TANGO2, is associated with impaired fatty acid oxidation and metabolic crises that frequently trigger QT prolongation and arrhythmias. The objective was to study the mechanisms responsible for QT prolongation and arrhythmogenesis in TDD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>CRISPR\/Cas9 were used to generate human-induced pluripotent stem cell cardiomyocytes (hiPSC-CMs) carrying a TANGO2 exon 3\u20139 deletion (TANGO2\u2212\/\u2212). Bioenergetic function (mitochondrial oxygen consumption rate, intracellular ATP\/ADP ratio) and action potentials (APs) were studied under glucose- or palmitate-fueled conditions. This study reports a TDD clinical case of QT prolongation and arrhythmia that responded favourably to L-type calcium channel (LTCC) inhibition with verapamil or vitamin B-complex supplementation. The mechanisms underlying clinical presentations and therapeutic responses were investigated using TANGO2\u2212\/\u2212 hiPSC-CMs. In glucose-containing medium, the bioenergetic function was comparable between control and TANGO2\u2212\/\u2212 hiPSC-CMs. In contrast, using palmitate as energy substrate triggered a profound reduction in cellular ATP production rate and decreased ATP\/ADP ratios in TANGO2\u2212\/\u2212 hiPSC-CMs, exacerbated by 24-h fasting. This crisis was prevented by 2-week treatment with vitamins B5 and B9. During the crisis, TANGO2\u2212\/\u2212 hiPSC-CMs exhibited AP prolongation, prevented by intracellular delivery of Mg-ATP or creatine kinase. LTCC inhibition with verapamil prevented AP prolongation by normalizing ATP\/ADP ratios and intracellular Ca mishandling. Importantly, the metabolic crisis upregulated TRPM4, an ATP- and Ca-regulated channel. TRPM4 siRNA knockdown or pharmacological block prevented AP prolongation without rescuing the energetic deficit of TANGO2\u2212\/\u2212 hiPSC-CMs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>These findings suggest a mechanistic link between ATP deficiency, TRPM4 activation, and AP prolongation in TDD. Targeting TRPM4 therapeutically may help prevent QT prolongation and cardiac arrhythmias in TDD crisis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/cvr\/cvag143","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T15:28:57Z","timestamp":1782919737000},"page":"1626-1641","source":"Crossref","is-referenced-by-count":0,"title":["Metabolic crisis and TRPM4 activation cause QT prolongation in TANGO2 deficiency disorder"],"prefix":"10.1093","volume":"122","author":[{"given":"Lili","family":"Wang","sequence":"first","affiliation":[{"name":"Vanderbilt Center for Arrhythmia Research and Therapeutics, Division of Clinical Pharmacology, Vanderbilt University Medical Center , 2215B Garland Ave, Nashville, TN 37232-0575, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyungsoo","family":"Kim","sequence":"additional","affiliation":[{"name":"Vanderbilt Center for Arrhythmia Research and Therapeutics, Division of Clinical Pharmacology, Vanderbilt University Medical Center , 2215B Garland Ave, Nashville, TN 37232-0575, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prince J","family":"Kannankeril","sequence":"additional","affiliation":[{"name":"Thomas P. Graham Jr. Division of Pediatric Cardiology and the Center for Pediatric Precision Medicine, Department of Pediatrics, Monroe Carell Jr. Children\u2019s Hospital at Vanderbilt and Vanderbilt University Medical Center , Nashville, TN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chen","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Vanderbilt University , Nashville, TN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel J","family":"Blackwell","sequence":"additional","affiliation":[{"name":"Vanderbilt Center for Arrhythmia Research and Therapeutics, Division of Clinical Pharmacology, Vanderbilt University Medical Center , 2215B Garland Ave, Nashville, TN 37232-0575, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel J","family":"Mackenzie","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Rochester Medicine , Rochester, NY, 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