Fitzl G, Meyer U, Wassilew G, Welt K
Institute of Anatomy, University of Leipzig, Germany.
Exp Toxicol Pathol. 1998 Jun;50(3):245-52. doi: 10.1016/S0940-2993(98)80092-4.
The hereditary cardiomyopathy of the Syrian hamster (strain BIO 8262) as a model of human cardiomyopathy was investigated during postnatal development using ultrastructural morphometric analysis. The aim of the study was to establish basic ultrastructural morphometric data of cardiomyocytes of this model during all stages of the disease in comparison to corresponding stages of normal development, and to compare the ultrastructural resistance of normal and myopathic myocardium against acute hypoxic stress. The postnatal development of this cardiomyopathy is characterized by focal myolytic lesions already in the first postnatal days, extended myofibrillic and mitochondrial damage developing after the 1st month, and features of hypertrophy, disarray of myofibrils, thickening, and clumping of z-lines after the 4th month. Morphometric parameters of myopathic cardiomyocytes differing from those of the control are diminished volume density of myofibrils by about 10% of the control values in middle age, slightly increased volume density of sarcoplasmic reticulum and t-tubules in the 1st months, significant increase of them after the 3rd month, and significant alterations of many mitochondrial parameters indicating degenerations (numeric density, volume density, average volume, volume density of cristae, and of destroyed areas, and ratio of surface to volume density of mitochondrial cristae). Acute isobaric hypoxia was applied to myopathic and healthy hamsters of corresponding age (at day 100). Evaluation of ultrastructural morphometric parameters of cardiomyocytes revealed that especially mitochondria were more affected by hypoxia in the myopathic animals. The average mitochondrial volume was significantly more increased in the myopathic condition, the ratio of surface to volume density of cristae significantly diminished. Our ultrastructural - morphometric study has shown, that morphometric parameters in general can be assigned to the stages of cardiomyopathy, and that the ultrastructural differences are best expressed about the 4th month.
利用超微结构形态计量分析,对叙利亚仓鼠(BIO 8262品系)的遗传性心肌病作为人类心肌病模型在出生后发育过程中进行了研究。本研究的目的是建立该模型心肌细胞在疾病各阶段的基本超微结构形态计量数据,并与正常发育的相应阶段进行比较,同时比较正常和病变心肌对急性缺氧应激的超微结构抵抗力。这种心肌病的出生后发育特征为:出生后最初几天就出现局灶性肌溶解病变,出生后1个月后出现广泛的肌原纤维和线粒体损伤,4个月后出现肥大、肌原纤维排列紊乱、Z线增粗和聚集等特征。与对照组相比,病变心肌细胞的形态计量参数有所不同,中年时肌原纤维的体积密度降低约为对照值的10%,出生后第1个月肌浆网和T小管的体积密度略有增加,第3个月后显著增加,许多线粒体参数发生显著改变,表明出现退化(数量密度、体积密度、平均体积、嵴的体积密度、破坏区域以及线粒体嵴的表面积与体积密度之比)。对相应年龄(第100天)的病变和健康仓鼠施加急性等压缺氧。对心肌细胞超微结构形态计量参数的评估表明,病变动物的心肌细胞中尤其是线粒体受缺氧影响更大。病变状态下线粒体的平均体积显著增加,嵴的表面积与体积密度之比显著降低。我们的超微结构形态计量研究表明,一般形态计量参数可与心肌病的阶段相对应,超微结构差异在大约第4个月时表现最为明显。