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电子期刊 >> 正文 |
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摘要：
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肥厚型心肌病(Hypertrophic cardiomyopathy,HCM)是一种不明原因的心肌肥厚,心肌纤维排列紊乱为特征的心肌病.它的发病率为0.04%～0.4%[1].它可以表现为多样的临床症状,是青少年患者发生心脏性猝死的常见原因,大部分患者以家族性常染色体显性遗传的规律发生,具有基因的异质性.本文综述了目前 HCM 发病机理的研究进展情况,重点阐述了β肌球蛋白的突变及定位、与临床表现及预后之间的关系、心脏肌钙蛋白 T、肌钙蛋白 I 和α-原肌球蛋白的突变定位、还有环境因素及一些原癌基因对 HCM 的作用.
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参考文献：
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Rayment I.Holden HM.Sellers JR Structural interpretation of the mutations in beta cardiac myosin that have been implicated in familial hypertrophic cardiomyopathy 1995 Ali J.Marian MD Sudden cardiac death in patients with hypertrophic cardiomyopathy:from bench to bedside with an emphasis on genetic matkers\^ 1995 Jarcho JA.Mckenna W.PateRPJ Mapping of a gene foRfamilial hypertrophic cardiomyopathy to chromosome 14q1 1989 AJ Marian MD.RRoherts MD MoleculaRgenetics of hypertropic cardiomyopathy 1995 Bemad NG.Vijak M MoleculaRbasis of cardiac performancePlasticity of the myocardium generated through grotein isoform switches 1989 ThierfeldeRL.Watkins H.Macrae C Alpha tropomyosin and cardiac troponin T mutations cause familial hypertrophic cardiomyopthy:a disease of the saromere 1994 RJohn Solar.Helen M.Rarick Troponin and tropomyosin proteins that switch on and tune in the activity of cardiac myofilaments 1998 Larry S.Tabacman Thin filament mediated regulation of cardiac contraction 1996 Annc M.Murphy H.Dimitrios G Transgenic mouse model of stunned myocardium 2000 Kathryn E.Hugh W.Charless R Altered regulatory propertics of human cardiac troponin I mutants that cause hypertrothic cardiomyopathy 2000 Watkins H.ThierfeldeRL.Hwang DS Sporadic hypertrophic cardiomyopathy:due to de novo myosin mutations 1992 况少青.于金德.陆林 肥厚型心肌病患者心脏β-肌球蛋白重链基因点突变的研究 1996 Akira M.Naohiro O.Ryosuke N Apieal hypertropic cardiomyopathy and hepatitisc virus infection 1999 汤健.周爱儒 原癌基因与心血管疾病 1990 Komuro I.Kurabayashim Expression of celluaRoncogenes in myocardium during the developmental stage and pressure overloaded hypertrophy of type rat heart 1988 Mulragh SL.Micheal LH.Perryman MB A hemodynamic load in vivo induces cardiac expression 1987 George coopeRIV MD Basic determinants of myocardial hypertrophy:A review of moleculaRmechanims 1997
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