Lucas-Héron B, Schmitt N, Ollivier B
Laboratoire de Physiologie, U.E.R. de Médecine, Nantes et Unité CNRS, France.
J Neurol Sci. 1990 Nov;99(2-3):349-53. doi: 10.1016/0022-510x(90)90169-n.
In our study, mitochondria were isolated from skeletal muscle in 3-, 5-, 6- and 16-week-old mdx and control mice. A deficit was observed in a calcium-specific mitochondrial protein (named "calmitine") in 3-, 5- and 6-week-old mdx mice but only in 3-week-old control mice. In addition, there was a correlation between the amounts of calmitine and calcium uptake in mitochondria: the latter remained low in 3-, 5- and 6-week-old mdx mice and was similar to controls in 16-week-old mdx mice (as was calmitine). A relationship is suggested between the deficit in calmitine (and calcium uptake in mitochondria) and the important signs of fiber degeneration presented by mdx mice between 3 and 6 weeks of age (a return to normal was observed subsequently).
在我们的研究中，从3周龄、5周龄、6周龄和16周龄的mdx小鼠及对照小鼠的骨骼肌中分离出线粒体。在3周龄、5周龄和6周龄的mdx小鼠中观察到一种钙特异性线粒体蛋白（名为“钙调素”）存在缺陷，但仅在3周龄的对照小鼠中存在该缺陷。此外，钙调素的量与线粒体中钙摄取之间存在相关性：在3周龄、5周龄和6周龄的mdx小鼠中，后者（钙摄取）保持在低水平，而在16周龄的mdx小鼠中与对照相似（钙调素也是如此）。这表明钙调素缺陷（以及线粒体中钙摄取）与mdx小鼠在3至6周龄时出现的纤维变性的重要迹象之间存在关联（随后观察到恢复正常）。
