Inverse correlation between the formation of mitochondria-derived vacuoles and Lewy-body-like hyaline inclusions in G93A superoxide-dismutase-transgenic mice
Abstract
In G93A mice, the most popular model of amyotrophic lateral sclerosis (ALS), neuronal Lewy-body-like hyaline inclusions (LBHIs) and mitochondria-derived vacuoles are observed in addition to motor neuron loss. Although LBHIs are thought to be toxic, the significance of the mitochondria-derived vacuoles has not been fully investigated. In this study, the relationship between the formation of these vacuoles and LBHIs was clarified statistically in the lumbar segment from two phyletic lines of G93A mice (G1L, G1H), using immunohistochemical methods. Furthermore, the distributions of vacuoles and LBHIs were examined in the pons including the facial nucleus, where pathological changes occur in ALS patients and G93A mice. Numerous vacuoles 2-3 microm in diameter were detected in the neuropil of the lumbar segment from G1L mice euthanatized approximately 3.5 months prior to the onset of the disease. Most of the vacuoles disappeared, but some became larger as the disease progressed. The number of vacuoles with a diameter exceeding 5 microm began to decrease after disease onset, while that of intra-neuritic LBHIs increased rapidly. There was a strong inverse correlation between the numbers of vacuoles and LBHIs in symptomatic mice (P<0.0...Continue Reading
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