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

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

Research output: Contribution to journalArticlepeer-review

26 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)2500-2505
Number of pages6
JournalFEBS Letters
Volume587
Issue number16
DOIs
Publication statusPublished - 2013 Aug 19

Keywords

  • ALS
  • Amyloid
  • Neurodegenerative disease
  • Protein aggregation
  • SOD1

ASJC Scopus subject areas

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

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