＜Poster＞Cystatin C immunoreactivity and neuronal degeneration in amyotrophic lateral sclerosis

概要

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Cystatin C （CC）, a cysteine protease inhibitor involved in protein degradation, is a marker of Buninabodies in lower motor neurons in amyotrophic lateral sclerosis （ALS）. TDP-43-immunoreactive inclusions are alsohistological hallmark of ALS. However, immunohistochemical localization of CC in ALS motor neurons with or withoutinclusions is uncertain. Recently, we demonstrated that the majority of anterior horn cells showed moderate to intenseimmunoreactivity for CC in controls and that CC immunoreactivity was severely decreased in anterior horn cellsin ALS. The proportion of CC-immunolabeled anterior horn cells was reduced regardless of whether those neuronscontained Bunina bodies or not. In contrast, the proportion of CC-immunolabeled anterior horn cells was signifi cantlyreduced owing to the presence of TDP-43 inclusions. These fi ndings suggest that the formation of TDP-43 inclusions,but not of Bunina bodies, reduces the content of CC in spinal motor neurons and that perturbations in endogenouslevels of CC in neurons may participate in neurodegenerative process in ALS.
弘前大学の論文