Bornman L, Polla B S, Lotz B P, Gericke G S
Department of Human Genetics and Developmental Biology, University of Pretoria.
Muscle Nerve. 1995 Jan;18(1):23-31. doi: 10.1002/mus.880180105.
Heat-shock/stress proteins (HSPs) are induced in response to stressful conditions and are essential for survival during and after cellular stress. We investigated whether dystrophin deficiency in muscle from Duchenne muscular dystrophy (DMD) patients induces HSPs. Immunohistochemical studies were performed on cryosections from normal muscle, heat-shocked muscle, and muscle from patients with DMD, dermatomyositis, and mitochondrial myopathy using antibodies against HSP 72/73, HSP 72, HSP 90, groEL (HSP 65 homologue), and ubiquitin. Computer-assisted image processing revealed a significant (P < 0.05) induction of HSP 72/73, 72, 65, and ubiquitin in hypercontracted fibers; HSP 90 and ubiquitin in regenerating fibers; and ubiquitin in macrophage invaded necrotic fibers of DMD muscle. No significant induction of HSPs was observed in dermatomyositis or mitochondrial myopathies. The stress response induced in DMD may relate to the metabolic stress characteristic of the disease and could represent an autoprotective mechanism. Manipulation of this protective response may reduce injury and have potential therapeutic application.
热休克/应激蛋白（HSPs）在应激条件下被诱导产生，对于细胞应激期间及之后的存活至关重要。我们研究了杜氏肌营养不良症（DMD）患者肌肉中的肌营养不良蛋白缺乏是否会诱导热休克蛋白产生。使用针对HSP 72/73、HSP 72、HSP 90、groEL（HSP 65同源物）和泛素的抗体，对正常肌肉、热休克肌肉以及患有DMD、皮肌炎和线粒体肌病患者的肌肉冰冻切片进行了免疫组织化学研究。计算机辅助图像处理显示，在过度收缩的纤维中，HSP 72/73、72、65和泛素有显著（P < 0.05）诱导；在再生纤维中，HSP 90和泛素有诱导；在DMD肌肉巨噬细胞侵入的坏死纤维中，泛素有诱导。在皮肌炎或线粒体肌病中未观察到热休克蛋白的显著诱导。DMD中诱导的应激反应可能与该疾病的代谢应激特征有关，并且可能代表一种自我保护机制。对这种保护反应的操控可能会减少损伤并具有潜在的治疗应用价值。
