心肌细胞骨架蛋白在终末期扩张型心肌病患者中的表达  

Abnormal myocardial cytoskeletal proteins in patients with end-stage dilated cardiomyopathy

作　　者：秦胜梅[1] 王齐兵[1] 庄亚敏[1] 李高平[1] 王春生[1] 陈昊[1] 杨英珍[1] 葛均波[1] 陈灏珠[1] 

机构地区：[1]复旦大学附属中山医院心内科上海市心血管病研究所,上海200032

出　　处：《临床心血管病杂志》2007年第7期484-487,共4页Journal of Clinical Cardiology

摘　　要：目的：探讨扩张型心肌病（DCM）及其他终末期心脏病患者衰竭心肌的细胞骨架蛋白actin、desmin、sarcoglycans（SGs）的表达情况。方法：9例终末期DCM（DCM组）与6例其他终末期心脏病患者和1例非心源性脑死亡者（对照组）的心肌标本进行免疫组化检测。结果：免疫组化检查结果显示，DCM组中8例存在上述多个骨架蛋白表达异常，且均有至少1个SGs亚型异常，其中5例伴有actin和（或）desmin的表达异常，心功能Ⅲ-Ⅳ级者骨架蛋白的异常（〉2个）较心功能Ⅱ-Ⅲ级者（≤2个）多；对照组中4例心功能Ⅳ级者也有细胞骨架蛋白表达异常，包括α、δ-SG异常各1例（分别为心脏肉瘤，缺血性心肌病），actin异常1例（先天性心脏病），以及α、β-SG及actin均异常1例（呈DCM表现的终末期非梗阻性肥厚型心肌病）；1例脑死亡者心肌中未检出上述骨架蛋白表达异常。结论：DCM衰竭心肌中存有多种细胞骨架蛋白的表达异常，多数为actin、desmin、SGs表达水平的降低，提示由细胞骨架蛋白的表达异常所致的细胞内外力的传递缺陷可能是导致终末期心脏病的共同发病或演进机制。Objective：To investigate myocardial cytoskeletal proteins including actin, desmin, sarcoglycans（α,β,γ,δ） in patients with dilated cardiomyopathy （DCM） and other end-stage heart diseases. Method：Nine patients （6 males, 3 females, aged [34±16]years） with advanced DCM and 6 patients （5 males, 1 female, aged [33±22] years） with other late-stage heart diseases including 2 cases of hypertrophic cardiomyopathy, 1 ischemic cardiomyopathy, 1 myocardial sarcoma, and 2 congenital heart diseases, and 1 non-cardiac brain-death patient were examined on left ventricular tissues by immunohistochemical study. Result： Immunohistochemical study showed 8 of 9 patients with DCM were found abnormal myocardial cytoskeletal proteins （actin, desmin, sarcoglycans）, manifesting disruption or reduction of cytoskeletal protein, in which at least 1 sub-type of sarcoglycans was abnormally expressed in the 8 patients, meanwhile, abnormal actin and/or desmin expression were demonstrated in 5 patients. Whereas, 4 of 6 non-DCM subjects, with NYHA grade Ⅳ showed abnormal cytoskeletal proteins including 1 case of α-sarcoglycan, 1 case of δ- sarcoglycan,1 case of actin and α,β- sarcoglycan, and 1 case of actin abnormality. As control, the non-cardiac brain death patient was found normal cytoskeletal protein. Conclusion： Abnormal cytoskeletal proteins like actin, desmin, sarcoglycans were expressed with reduction or disruption in failing myocardium DCM and other end-stage heart diseases, indicating that transmembrane force transmitting defects caused by abnormal cytoskeletal proteins mightbe the common underlying pathogenesis for end-stage heart diseases.

关 键 词：心肌病 扩张型 心力衰竭 充血性 骨架蛋白 

分 类 号：R542.2[医药卫生—心血管疾病]