Takemura G, Takatsu Y, Sakaguchi H, Fujiwara H
Department of Internal Medicine, Hyogo Prefectural Amagasaki Hospital, Japan.
Pathol Res Pract. 1997;193(4):305-11. doi: 10.1016/s0344-0338(97)80008-8.
Intranuclear mitochondria have occasionally been reported in various cells including cardiac myocytes, but the incidence and details including the specific mechanism(s) whereby mitochondria exist in the nuclear matrix are unknown. We studied the ultrastructure of a total of 91 consecutive endomyocardial biopsy specimens obtained from 50 patients with various cardiac diseases. Intranuclear mitochondria were found in three patients: two with hypertrophic cardiomyopathy and one with chronic myocarditis. Their myocytes were markedly hypertrophic and the shapes of the nuclei were bizarre. Specimens from these patients were further studied on serial sections, and some of the nuclei with mitochondria showed ruptured nuclear envelope. Intranuclear mitochondria were found in at least 6% of the patients with cardiac diseases (3% of the specimens examined), and a novel mechanism of the pathogenesis was documented: communication between the nuclear matrix and cytoplasm through the rupture in the nuclear envelope. The nuclear rupture might be simply due to a laboratory artifact. On the other hand, it might imply some pathophysiological significances in the degeneration of myocytes in cardiac diseases.
包括心肌细胞在内的各种细胞中偶尔有关于核内线粒体的报道,但线粒体存在于核基质中的发生率及细节,包括具体机制尚不清楚。我们研究了从50例患有各种心脏疾病的患者中获取的共91份连续心内膜心肌活检标本的超微结构。在3例患者中发现了核内线粒体:2例肥厚型心肌病患者和1例慢性心肌炎患者。他们的心肌细胞明显肥大,细胞核形态怪异。对这些患者的标本进行连续切片进一步研究,一些含有线粒体的细胞核显示核膜破裂。在至少6%的心脏病患者(所检查标本的3%)中发现了核内线粒体,并记录了一种新的发病机制:核基质与细胞质通过核膜破裂进行通讯。核破裂可能仅仅是由于实验室假象。另一方面,它可能意味着在心脏病心肌细胞变性中存在一些病理生理意义。