Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis

Yoshiaki Furukawa, Kumi Kaneko, Shoji Watanabe, Koji Yamanaka, Nobuyuki Nukina

研究成果: Article査読

28 被引用数 (Scopus)

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.

本文言語English
ページ(範囲)2500-2505
ページ数6
ジャーナルFEBS Letters
587
16
DOI
出版ステータスPublished - 2013 8 19

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Cell Biology

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