{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T13:12:58Z","timestamp":1790082778748,"version":"4.0.1"},"reference-count":56,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["22\u201014\u201000059\u2010P"],"award-info":[{"award-number":["22\u201014\u201000059\u2010P"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cytoskeleton"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Tropomyosin is a highly conserved \u03b1\u2010helical coiled\u2010coil protein that is involved in maintaining the structural integrity of the sarcomere and regulating contraction of striated muscle. Mutations in the\n                    <jats:italic>TPM1<\/jats:italic>\n                    gene encoding cardiac \u03b1\u2010tropomyosin (Tpm1.1) are associated with left ventricular noncompact cardiomyopathy and congenital heart defects (CHD). We investigated the structural and functional properties of Tpm1.1 with the I130V and S229F mutations associated with CHD. We found that both mutations decreased the Tpm affinity for F\u2010actin and differently affected the sliding velocity of F\u2010actin\u2010Tpm filaments over ventricular and atrial myosin in an in\u00a0vitro motility assay. The results indicate a disruption in the activation of F\u2010actin\u2010Tpm filaments by myosin cross\u2010bridges with both atrial and ventricular myosin. The I130V mutation increased the maximum sliding velocity of thin filaments over atrial myosin and decreased the Ca\n                    <jats:sup>2+<\/jats:sup>\n                    sensitivity of the filament velocity. The S229F mutation increased the maximum filament velocity on atrial myosin and decreased the Hill coefficient. The mutations did not affect the Ca\n                    <jats:sup>2+<\/jats:sup>\n                    \u2010dependent characteristics of thin filament velocity with ventricular myosin. Thus, the molecular mechanism of CHD pathogenesis induced by Tpm1.1 mutations is associated with changes in sarcomere stability and disruption of actin\u2010myosin interaction in the myocardium of the ventricles and atria, which negatively affect the contractility of both heart chambers.\n                  <\/jats:p>","DOI":"10.1002\/cm.70206","type":"journal-article","created":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T12:35:43Z","timestamp":1790080543000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The\n                    <scp>I130V<\/scp>\n                    and\n                    <scp>S229F<\/scp>\n                    Tropomyosin Mutations Associated With Congenital Heart Disease Disrupt Actin\u2010Myosin Interactions in the Atria"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2976-2108","authenticated-orcid":false,"given":"Galina V.","family":"Kopylova","sequence":"first","affiliation":[{"name":"Institute of Immunology and Physiology, Ural Branch of the Russian Academy of Sciences  Yekaterinburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5583-5342","authenticated-orcid":false,"given":"Anastasia M.","family":"Kochurova","sequence":"additional","affiliation":[{"name":"Institute of Immunology and Physiology, Ural Branch of the Russian Academy of Sciences  Yekaterinburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrey V.","family":"Slushchev","sequence":"additional","affiliation":[{"name":"Bach Institute of Biochemistry, Research Center of Biotechnology of the Russian Academy of Sciences  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daria S.","family":"Yampolskaya","sequence":"additional","affiliation":[{"name":"Bach Institute of Biochemistry, Research Center of Biotechnology of the Russian Academy of Sciences  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-6418-8980","authenticated-orcid":false,"given":"Julia Y.","family":"Antonets","sequence":"additional","affiliation":[{"name":"Institute of Immunology and Physiology, Ural Branch of the Russian Academy of Sciences  Yekaterinburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia S.","family":"Ryabkova","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Faculty of Biology Lomonosov Moscow State University  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5939-6201","authenticated-orcid":false,"given":"Ivan A.","family":"Katrukha","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Faculty of Biology Lomonosov Moscow State University  Moscow Russia"},{"name":"HyTest Ltd.  Turku Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5085-0327","authenticated-orcid":false,"given":"Sergey Y.","family":"Kleymenov","sequence":"additional","affiliation":[{"name":"Bach Institute of Biochemistry, Research Center of Biotechnology of the Russian Academy of Sciences  Moscow Russia"},{"name":"Koltzov Institute of Developmental Biology of the Russian Academy of Sciences  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5342-1739","authenticated-orcid":false,"given":"Sergey Y.","family":"Bershitsky","sequence":"additional","affiliation":[{"name":"Institute of Immunology and Physiology, Ural Branch of the Russian Academy of Sciences  Yekaterinburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2645-4593","authenticated-orcid":false,"given":"Alexander M.","family":"Matyushenko","sequence":"additional","affiliation":[{"name":"Bach Institute of Biochemistry, Research Center of Biotechnology of the Russian Academy of Sciences  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7755-9895","authenticated-orcid":false,"given":"Dmitrii I.","family":"Levitsky","sequence":"additional","affiliation":[{"name":"Bach Institute of Biochemistry, Research Center of Biotechnology of the Russian Academy of Sciences  Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6660-4673","authenticated-orcid":false,"given":"Daniil V.","family":"Shchepkin","sequence":"additional","affiliation":[{"name":"Institute of Immunology and Physiology, Ural Branch of the Russian Academy of Sciences  Yekaterinburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,22]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.17.2507"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi101632f"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj3270335"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0509269102"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.abb.2013.12.021"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00519.2015"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1529\/biophysj.105.064634"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2005.084194"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehm342"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yjmcc.2017.03.006"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yjmcc.2012.07.018"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.14.7836"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/bip.1978.360170212"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2003.058487"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2004.070631b"},{"issue":"8","key":"e_1_2_8_17_1","doi-asserted-by":"crossref","DOI":"10.1152\/ajpheart.00528.2014","article-title":"The Functional Effect of Dilated Cardiomyopathy Mutation (R144W) in Mouse Cardiac Troponin T Is Differently Affected by \u03b1\u2010 and \u03b2\u2010Myosin Heavy Chain Isoforms","volume":"308","author":"Gollapudi S. K.","year":"2015","journal-title":"American Journal of Physiology. Heart and Circulatory Physiology"},{"key":"e_1_2_8_18_1","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1007\/978-1-4684-6039-1_22","article-title":"Skeletal Muscle Regulatory Proteins Enhance F\u2010Actin in Vitro Motility","volume":"453","author":"Gordon A. M.","year":"1998","journal-title":"Advances in Experimental Medicine and Biology"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2011.08.043"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurjhf\/hfq225"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469\u20107793.2000.00233.x"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006\u20103495(03)74543\u20103"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2021.12.051"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1134\/S0006297913030073"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/227680a0"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.44.31279"},{"key":"e_1_2_8_27_1","doi-asserted-by":"crossref","first-page":"14051","DOI":"10.1074\/jbc.272.22.14051","article-title":"The Active State of the Thin Filament Is Destabilized by an Internal Deletion in Tropomyosin","volume":"272","author":"Landis C. A.","year":"1997","journal-title":"Journal of Biological Chemistry"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)34298-4"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2006.088229"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076\u20106879(82)85009\u2010x"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1529\/biophysj.106.081117"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/febs.12756"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijbiomac.2018.09.105"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1021\/acs.biochem.6b00994"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/dvdy.24115"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00487.2016"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0021\u20109258(17)34082\u20106"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehi025"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms232415723"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/13837"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076\u20106879(82)85020\u20109"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M204749200"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2011.10.022"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2017.06.043"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1002\/cm.20513"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2012.05.095"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1093\/europace\/eup187"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M707485200"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41390\u2010018\u20100162\u20101"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.xcrm.2021.100501"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1038\/415227a"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms24010018"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2012.11.003"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.12.019"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.22.12488"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardiores.2003.09.011"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms252313059"}],"container-title":["Cytoskeleton"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cm.70206","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cm.70206","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cm.70206","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T12:35:45Z","timestamp":1790080545000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/cm.70206"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,22]]},"references-count":56,"alternative-id":["10.1002\/cm.70206"],"URL":"https:\/\/doi.org\/10.1002\/cm.70206","archive":["Portico"],"relation":{},"ISSN":["1949-3584","1949-3592"],"issn-type":[{"value":"1949-3584","type":"print"},{"value":"1949-3592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,22]]},"assertion":[{"value":"2026-07-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-11","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-22","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70206"}}