@article{oai:niigata-u.repo.nii.ac.jp:00009489,
 author = {豊島, 靖子},
 issue = {12},
 journal = {新潟医学会雑誌, 新潟医学会雑誌},
 month = {Dec},
 note = {Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disorder. One of the neuropathological hallmarks of ALS is the presence of ubiquitinated neuronal cytoplasmic inclusions (NCIs) in lower motor neurons. Recently, the 43kDa TARDNA-binding protein (TDP-43) has been identified as the major component of NCIs in sporadic ALS. While initially thought to be relatively specific to ALS and fronto-temporal lober degeneration (FTLD)-TDP, TDP-43 pathology has now been detected in a number of other neurodegenerative diseases, many associated with tau pathology, including Alzheimer's disease (AD). However, in these diseases, TDP-43 related neurodegenerative process may occur in association with other distinct pathologic processes (i.e. secondary TDP-43 proteinopathies). We recently reported TDP-43 pathology in a patient of spinocerebellar ataxia type 2 (SCA2), which is one of the polyglutamine diseases. Recently, intermediate-length polyglutamine (polyQ) expansions (27-33 Qs) in ataxin 2 (candidate gene of SCA2) is reported as a genetic risk factor for sporadic ALS. We discussed about the relations of ALS and other neurodegenerative diseases, from the pathological findings in TDP-43 proteinopathy.},
 pages = {647--654},
 title = {筋萎縮性側索硬化症と近縁疾患の病理所見 : TDP-43 proteinopathyのひろがりについて},
 volume = {126},
 year = {2012}
}