@article{oai:hirosaki.repo.nii.ac.jp:00003709,
 author = {Mori, Fumiaki and Tanji, Kunikazu and Miki, Yasuo and Wakabayashi, Koichi},
 issue = {Supplement},
 journal = {弘前医学},
 month = {Jul},
 note = {Cystatin C （CC）, a cysteine protease inhibitor involved in protein degradation, is a marker of Bunina bodies in lower motor neurons in amyotrophic lateral sclerosis （ALS）. TDP-43-immunoreactive inclusions are also histological hallmark of ALS. However, immunohistochemical localization of CC in ALS motor neurons with or without inclusions is uncertain. Recently, we demonstrated that the majority of anterior horn cells showed moderate to intense immunoreactivity for CC in controls and that CC immunoreactivity was severely decreased in anterior horn cells in ALS. The proportion of CC-immunolabeled anterior horn cells was reduced regardless of whether those neurons contained Bunina bodies or not. In contrast, the proportion of CC-immunolabeled anterior horn cells was signifi cantly reduced 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 endogenous levels of CC in neurons may participate in neurodegenerative process in ALS., 弘前医学. 61(Suppl.), 2010, p.S211-S214},
 pages = {S211--S214},
 title = {＜Poster＞Cystatin C immunoreactivity and neuronal degeneration in amyotrophic lateral sclerosis},
 volume = {61},
 year = {2010}
}