Fidziańska Anna, Walczak Ewa, Szwoch Małgorzata
Neuromuscular Unit, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland.
Ultrastruct Pathol. 2013 Feb;37(1):52-5. doi: 10.3109/01913123.2011.614719. Epub 2011 Oct 7.
Ultrastructural analysis was performed in cardiac ceroidlipofuscinosis to confirm the presence and the nature of storage material. Granular osmophilic deposits characteristic of GROD structures coincidented with particularly aberrant mitochondria. Remodeling of mitochondrial interior with the appearance of several form of abnormal inclusions was never observed in cardiac ceroidlipofuscinosis. The presence of dense osmophilic bodies, glycogen conglomerates, balloon-like and onion-like structures in mitochondrial interior seem to be early events of this storage process.
对心脏蜡样脂褐质沉积症进行了超微结构分析，以确认储存物质的存在及其性质。具有GROD结构特征的颗粒状嗜锇沉积物与特别异常的线粒体同时出现。在心脏蜡样脂褐质沉积症中从未观察到线粒体内出现多种异常包涵体形式的线粒体内部重塑。线粒体内存在致密嗜锇小体、糖原聚集体、气球样和洋葱样结构似乎是这种储存过程的早期事件。
