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目的探讨扩张型心肌病(DCM)以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、α、β、γ、δ)表达情况。方法对9例终末期DCM与6例其他终末期心脏病患者和l例非心源性脑死亡者的心尖部心肌标本进行免疫荧光或免疫组化检测,采用Fisher’s精确概率检验法分析aCtin、desmin、SGs(α、β、γ、δ)在DCM组和对照组中表达异常的分布情况。结果免疫组化检查结果显示,8例DCM患者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γ-SG异常1例,α-SG异常1例,α-／γ-SG异常3例,而β-／δ-SG、γ-／δ-SG及α-／β-／γ-／δ-SG异常各1例,有5例患者还伴有actin和(或)desmin的表达异常;这8例患者术前心功能状况与受累骨架蛋白异常的数目相关,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;6例非DCM患者中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 the expression of actin, desmin and sarcoglycans (SGs, α, β, γ, δ) in patients with dilated cardiomyopathy (DCM) and other patients with end-stage heart disease. Methods Nine patients with end-stage DCM and six patients with other end-stage heart disease and one noncardiogenic brain-dead myocardium were examined by immunofluorescence or immunohistochemistry. Fisher’s exact test was used to analyze aCtin, desmin, SGs (α, β, γ, δ) in the DCM group and control group abnormal distribution of the distribution. Results The immunohistochemical results showed that there were abnormal expression of the above multiple scaffold proteins in 8 DCM patients and at least 1 subtype of SGs was abnormal. Among them, 1 was abnormal in γ-SG, 1 was abnormal in α-SG, There were 3 cases of γ-SG abnormalities, and 1 case of abnormalities of β- / δ-SG, γ- / δ-SG and α- / β- / γ- / δ- SG. ) desmin was abnormal. The preoperative cardiac function status was related to the number of abnormal skeletal proteins involved in the eight patients. The skeletal protein abnormalities (> 2) in patients with cardiac function Ⅲ-Ⅳ were more severe than those with cardiac function Ⅱ-Ⅲ 2). Six patients with non-DCM had abnormal cytoskeletal protein expression in four patients with cardiac function grade Ⅳ, including 1 case of abnormal α, δ-SG (cardiomyosarcoma and ischemic cardiomyopathy, respectively), abnormal actin 1 case (congenital heart disease), and 1 case of abnormal α, β-SG and actin (terminal non-obstructive hypertrophic cardiomyopathy showed DCM); 1 case of brain death did not detect the above-mentioned skeletal protein expression abnormal. Conclusions The expression of many cytoskeletal proteins in DCM-depleted myocardium is abnormal, most of which are actin, desmin and SGs, suggesting that the defect of intracellular and extracellular force transmission caused by the abnormal expression of cytoskeletal protein may be the cause of the end-stage Common pathogenesis or evolution of heart disease.