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Hokkaido University Collection of Scholarly and Academic Papers >
Graduate School of Life Science / Faculty of Advanced Life Science >
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Intracellular Conformation of Amyotrophic Lateral Sclerosis-Causative TDP-43
| Title: | Intracellular Conformation of Amyotrophic Lateral Sclerosis-Causative TDP-43 |
| Authors: | Kitamura, Akira Browse this author →KAKEN DB | | Yuno, Sachiko Browse this author | | Kawamura, Rintaro Browse this author | | Kinjo, Masataka Browse this author →KAKEN DB |
| Keywords: | TDP-43 | | amyotrophic lateral sclerosis | | protein structure | | Forster resonance energy transfer | | fluorescence correlation spectroscopy |
| Issue Date: | Mar-2023 |
| Publisher: | MDPI |
| Journal Title: | International Journal of Molecular Sciences |
| Volume: | 24 |
| Issue: | 6 |
| Start Page: | 5513 |
| Publisher DOI: | 10.3390/ijms24065513 |
| Abstract: | Transactive response element DNA/RNA-binding protein 43 kDa (TDP-43) is the causative protein of amyotrophic lateral sclerosis (ALS); several ALS-associated mutants of TDP-43 have been identified. TDP-43 has several domains: an N-terminal domain, two RNA/DNA-recognition motifs, and a C-terminal intrinsically disordered region (IDR). Its structures have been partially determined, but the whole structure remains elusive. In this study, we investigate the possible end-to-end distance between the N- and C-termini of TDP-43, its alterations due to ALS-associated mutations in the IDR, and its apparent molecular shape in live cells using Forster resonance energy transfer (FRET) and fluorescence correlation spectroscopy (FCS). Furthermore, the interaction between ALS-associated TDP-43 and heteronuclear ribonucleoprotein A1 (hnRNP A1) is slightly stronger than that of wild-type TDP-43. Our findings provide insights into the structure of wild-type and ALS-associated mutants of TDP-43 in a cell. |
| Type: | article |
| URI: | http://hdl.handle.net/2115/89322 |
| Appears in Collections: | 生命科学院・先端生命科学研究院 (Graduate School of Life Science / Faculty of Advanced Life Science) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 北村 朗
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