-
- Katsuno Masahisa
- Department of Neurology, Nagoya University Graduate School of Medicine
-
- Iguchi Yohei
- Department of Neurology, Nagoya University Graduate School of Medicine
-
- Sobue Gen
- Department of Neurology, Nagoya University Graduate School of Medicine
Bibliographic Information
- Other Title
-
- 運動ニューロンの老化と変性
- ウンドウ ニューロン ノ ロウカ ト ヘンセイ
Search this article
Description
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that selectively affects upper and lower motor neurons. TAR DNA-binding protein 43 kDa (TDP-43), an RNA-binding protein redistributes from nucleus to cytoplasm and forms aggregates, which are the histopathological hallmark of sporadic amyotrophic lateral sclerosis, in affected motor neurons. TDP-43 in the aberrant inclusion is known to be hyperphosphorylated at C-terminal sites, to be truncated at the N-terminal region, and to re- distribute from nucleus to cytoplasm or neurite. Ethacrynic acid (EA), which increases cellular oxidative stress through glutathione depletion, induces TDP-43C-terminal phosphorylation, insolubilization, C-terminal fragmentation, and cytoplasmic distribution in cultured neuronal cells and primary cortical neurons, suggesting that the loss of nuclear TDP-43 may play a role in the pathogenesis of ALS. Motor neuron- specific knock out of TDP- 43 results in progressive weight loss and motor impairment together with degeneration of large motor axon, grouped atrophy of the skeletal muscle, and denervation in the neuromuscular junction. These findings suggest that loss-of-function of TDP-43 appears to contribute to the pathogenesis of ALS.
Journal
-
- Japanese Journal of Biological Psychiatry
-
Japanese Journal of Biological Psychiatry 24 (4), 185-190, 2013
Japanese Society of Biological Psychiatry
- Tweet
Keywords
Details 詳細情報について
-
- CRID
- 1390001205412495232
-
- NII Article ID
- 130005395514
-
- NII Book ID
- AA12468060
-
- ISSN
- 21866465
- 21866619
-
- NDL BIB ID
- 025132214
-
- Text Lang
- ja
-
- Data Source
-
- JaLC
- NDL
- CiNii Articles
-
- Abstract License Flag
- Disallowed