Szczepanowska Joanna, Zabłocki Krzysztof, Duszyński Jerzy
Department of Cellular Biochemistry, Nencki Institute of Experimental Biology, Pasteura 3, 02 093 Warsaw, Poland.
FEBS Lett. 2004 Dec 17;578(3):316-22. doi: 10.1016/j.febslet.2004.11.022.
Effects of T8993G mutation in mitochondrial DNA (mtDNA), associated with neurogenical muscle weakness, ataxia and retinitis pigmentosa (NARP), on the cytoskeleton, mitochondrial network and calcium homeostasis in human osteosarcoma cells were investigated. In 98% NARP and rho(0) (lacking mtDNA) cells, the organization of the mitochondrial network and actin cytoskeleton was disturbed. Capacitative calcium entry (CCE) was practically independent of mitochondrial energy status in osteosarcoma cell lines. The significantly slower Ca(2+) influx rates observed in 98% NARP and rho(0), in comparison to parental cells, indicates that proper actin cytoskeletal organization is important for CCE in these cells.
研究了与神经源性肌肉无力、共济失调和色素性视网膜炎(NARP)相关的线粒体DNA(mtDNA)中的T8993G突变对人骨肉瘤细胞的细胞骨架、线粒体网络和钙稳态的影响。在98%的NARP和rho(0)(缺乏mtDNA)细胞中,线粒体网络和肌动蛋白细胞骨架的组织受到干扰。在骨肉瘤细胞系中,容量性钙内流(CCE)实际上与线粒体能量状态无关。与亲代细胞相比,在98%的NARP和rho(0)细胞中观察到的Ca(2+)内流速率明显较慢,这表明适当的肌动蛋白细胞骨架组织对这些细胞中的CCE很重要。