抄録
Once a protein adopts the fibrillar aggregate conformation, a seeding reaction becomes operative in which pre-formed fibrils function as seeds for soluble protein molecules to be fibrillized. Such a seeding reaction accelerates the protein fibrillation in vitro; however, more investigation is required to test the seeded fibrillation inside cells. Here, we show that in vitro Cu,Zn-superoxide dismutase (SOD1) fibrils are transduced into cells and function as seeds to trigger the aggregation of endogenously expressed SOD1. Seeded aggregation of mutant SOD1 will thus play roles in a molecular pathomechanism of SOD1-linked amyotrophic lateral sclerosis.
| 元の言語 | English |
|---|---|
| ページ（範囲） | 2500-2505 |
| ページ数 | 6 |
| ジャーナル | FEBS Letters |
| 巻 | 587 |
| 発行部数 | 16 |
| DOI | |
| 出版物ステータス | Published - 2013 8 19 |
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ASJC Scopus subject areas
- Biochemistry
- Biophysics
- Cell Biology
- Genetics
- Molecular Biology
- Structural Biology
これを引用
Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis. / Furukawa, Yoshiaki; Kaneko, Kumi; Watanabe, Shoji; Yamanaka, Koji; Nukina, Nobuyuki.
：: FEBS Letters, 巻 587, 番号 16, 19.08.2013, p. 2500-2505.研究成果: Article
}
TY - JOUR
T1 - Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis
AU - Furukawa, Yoshiaki
AU - Kaneko, Kumi
AU - Watanabe, Shoji
AU - Yamanaka, Koji
AU - Nukina, Nobuyuki
PY - 2013/8/19
Y1 - 2013/8/19
N2 - Once a protein adopts the fibrillar aggregate conformation, a seeding reaction becomes operative in which pre-formed fibrils function as seeds for soluble protein molecules to be fibrillized. Such a seeding reaction accelerates the protein fibrillation in vitro; however, more investigation is required to test the seeded fibrillation inside cells. Here, we show that in vitro Cu,Zn-superoxide dismutase (SOD1) fibrils are transduced into cells and function as seeds to trigger the aggregation of endogenously expressed SOD1. Seeded aggregation of mutant SOD1 will thus play roles in a molecular pathomechanism of SOD1-linked amyotrophic lateral sclerosis.
AB - Once a protein adopts the fibrillar aggregate conformation, a seeding reaction becomes operative in which pre-formed fibrils function as seeds for soluble protein molecules to be fibrillized. Such a seeding reaction accelerates the protein fibrillation in vitro; however, more investigation is required to test the seeded fibrillation inside cells. Here, we show that in vitro Cu,Zn-superoxide dismutase (SOD1) fibrils are transduced into cells and function as seeds to trigger the aggregation of endogenously expressed SOD1. Seeded aggregation of mutant SOD1 will thus play roles in a molecular pathomechanism of SOD1-linked amyotrophic lateral sclerosis.
KW - ALS
KW - Amyloid
KW - Neurodegenerative disease
KW - Protein aggregation
KW - SOD1
UR - http://www.scopus.com/inward/record.url?scp=84881481605&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84881481605&partnerID=8YFLogxK
U2 - 10.1016/j.febslet.2013.06.046
DO - 10.1016/j.febslet.2013.06.046
M3 - Article
C2 - 23831581
AN - SCOPUS:84881481605
VL - 587
SP - 2500
EP - 2505
JO - FEBS Letters
JF - FEBS Letters
SN - 0014-5793
IS - 16
ER -