Abstract
In the present study, we used the transgenic mice expressing a human Cu/Zn SOD mutation (SOD1 G93A) as an in vivo model of amyotrophic lateral sclerosis (ALS) and performed immunohistochemical studies to investigate the changes of poly(ADP-ribose) polymerase (PARP) in the central nervous system. In the spinal cord of symptomatic transgenic mice, immunohistochemistry showed intensely stained PARP-immunoreactive glial cells with the appearance of astrocytes, which were confirmed as astrocytes by double-immunofluorescences. In the brainstem and cerebellum, PARP-immunoreactive astrocytes were observed in the medullary and pontine reticular formation, hypoglossal nucleus, vestibular nucleus, cochlear nucleus and cerebellar nuclei. On the contrary, no PARP-immunoreactive glial cells were observed in control mice although PARP-immunoreactive motor neurons were found. In presymptomatic transgenic mice, a few moderately stained neurons were observed, whereas PARP-immunoreactive astrocytes were not detected. The present study provides the first evidence that PARP-immunoreactive astrocytes were found in the central nervous system of symptomatic SOD1 G93A transgenic mice, suggesting that reactive astrocytes may play an important role in the pathogenesis and progress of ALS.
Published Version
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