R6/2小鼠中亨廷顿肌肉线粒体对钙离子的稳定性较低。
Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.
作者信息
Gizatullina Zemfira Z, Lindenberg Katrin S, Harjes Phoebe, Chen Ying, Kosinski Christoph M, Landwehrmeyer Bernhard G, Ludolph Albert C, Striggow Frank, Zierz Stephan, Gellerich Frank N
机构信息
KeyNeurotek AG, ZENIT Technology Park, Magdeburg, Germany.
出版信息
Ann Neurol. 2006 Feb;59(2):407-11. doi: 10.1002/ana.20754.
OBJECTIVE
The aim of the present work was the detection of Mitochondrial dysfunction of Huntington's disease (HD).
METHODS
We investigated muscle and muscle mitochondria of 14- to 16-week-old R6/2 mice in comparison with wild-type mice.
RESULTS
Atrophic fibers, increased fuchsinophilic aggregates, and reduced cytochrome c oxidase (15%) were found in HD muscle. With swelling measurements and Ca2+ accumulation experiments, a decreased stability of HD mitochondria against Ca2+-induced permeability transition was detected. Complex I-dependent respiration of HD mitochondria was more sensitive to inhibition by adding 10 microm Ca2+ than wild-type mitochondria.
INTERPRETATION
Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy.
目的
本研究旨在检测亨廷顿舞蹈病（HD）的线粒体功能障碍。
方法
我们研究了14至16周龄的R6/2小鼠与野生型小鼠的肌肉及肌肉线粒体。
结果
在HD小鼠肌肉中发现了萎缩纤维、嗜品红聚集体增加以及细胞色素c氧化酶减少（15%）。通过肿胀测量和Ca2+积累实验，检测到HD线粒体对Ca2+诱导的通透性转变的稳定性降低。与野生型线粒体相比，HD线粒体的复合体I依赖性呼吸对添加10微摩尔Ca2+的抑制作用更敏感。
解读
数据表明，HD线粒体对Ca2+的稳定性降低导致了能量抑制和细胞萎缩。
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