{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T13:10:47Z","timestamp":1787317847068,"version":"build-2736575974"},"reference-count":83,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2007,4,20]],"date-time":"2007-04-20T00:00:00Z","timestamp":1177027200000},"content-version":"vor","delay-in-days":3396,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiovasc electrophysiol"],"published-print":{"date-parts":[[1998,1]]},"abstract":"<jats:p>\n                    Genetics of SCD in HCM. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease caused by mutations in sarcomeric proteins. The disease is characterized by left ventricular hypertrophy in the absence of an increased external load, and myofibrillar disarray. A large number of mutations in genes coding for the \u03b2\u2010myosin heavy chain (\u03b2\u2010MyHC), cardiac troponin T (cTnT), cardiac troponin I, \u03b1\u2010tropomyosin, myosin binding protein C (MyBP\u2010C), and myosin light chain 1 and 2 in patients with HCM have been identified. Genotype\u2010phenotype correlation studies have shown that mutations carry prognostic significance. The Gly\n                    <jats:sup>256<\/jats:sup>\n                    Glu, Val\n                    <jats:sup>606<\/jats:sup>\n                    Met, and Leu\n                    <jats:sup>908<\/jats:sup>\n                    Val mutations in the \u03bc\u2010MyHC are associated with a benign prognosis. In contrast, Arg\n                    <jats:sup>403<\/jats:sup>\n                    Gln, Arg\n                    <jats:sup>719<\/jats:sup>\n                    Trp, and Arg\n                    <jats:sup>453<\/jats:sup>\n                    Cys mutations are associated with a high incidence of sudden cardiac death (SCD). Mutations in cTnT are associated with a mild degree of hypertrophy, but a high incidence of SCD. Mutations in MyBP\u2010C are associated with mild hypertrophy and a benign prognosis. However, it has become evident that factors other than the underlying mutations, such as genetic background and possibly environmental factors, also modulate phenotypic expression of HCM.\n                  <\/jats:p>","DOI":"10.1111\/j.1540-8167.1998.tb00871.x","type":"journal-article","created":{"date-parts":[[2007,4,20]],"date-time":"2007-04-20T10:48:49Z","timestamp":1177066129000},"page":"88-99","source":"Crossref","is-referenced-by-count":88,"title":["Molecular Genetic Basis of Hypertrophic Cardiomyopathy:"],"prefix":"10.1111","volume":"9","author":[{"given":"ALI J.","family":"MARIAN","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ROBERT","family":"ROBERTS","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,4,20]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.92.5.1336"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.91.2.532"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.suppl_1.1721"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.45.4.769"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1996.03540030033028"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.92.4.785"},{"key":"e_1_2_1_8_2","first-page":"41","article-title":"Hypertrophic cardiomyopathy and its markers in the general population. 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