Hengstenberg C, Maisch B
Cardiology Department, Philipps-University of Marburg, Germany.
Cardioscience. 1993 Mar;4(1):15-20.
Hypertrophic cardiomyopathy is characterized by an unexplained hypertrophy of the left ventricle, particularly the interventricular septum. Although point mutations in the beta-myosin chain gene have been found in several US families in familiar hypertrophic cardiomyopathy, the pathogenetic pathways leading to myocyte hypertrophy, the most important feature, are still not clear. To examine whether activation (expression) of nuclear proto-oncogenes may play a role in hypertrophic cardiomyopathy, endomyocardial biopsies from 13 patients with hypertrophic cardiomyopathy were examined using monoclonal antibodies against c-myc, c-fos and c-jun. The nuclear proto-oncogenes c-fos, c-jun and c-myc were expressed in 53, 60, and 50%, respectively, of patients with hypertrophic cardiomyopathy. In control biopsies, c-myc was detectable in only 10% of the patients, while c-fos and c-jun were always undetectable. These results show that nuclear proto-oncogenes are induced in patients with hypertrophic cardiomyopathy, although the triggering mechanisms remain unknown.
肥厚型心肌病的特征是左心室，尤其是室间隔出现无法解释的肥厚。尽管在美国的几个家族性肥厚型心肌病家族中发现了β-肌球蛋白链基因的点突变，但导致心肌细胞肥大（这一最重要特征）的致病途径仍不清楚。为了研究核原癌基因的激活（表达）是否可能在肥厚型心肌病中起作用，使用针对c-myc、c-fos和c-jun的单克隆抗体对13例肥厚型心肌病患者的心内膜活检组织进行了检查。核原癌基因c-fos、c-jun和c-myc在分别53%、60%和50%的肥厚型心肌病患者中表达。在对照活检中，仅10%的患者可检测到c-myc，而c-fos和c-jun始终无法检测到。这些结果表明，肥厚型心肌病患者的核原癌基因被诱导，尽管触发机制仍然未知。
