@article{1dbc9ec8384e48c4b13808e9cbb15bd0,
title = "Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis",
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.",
keywords = "ALS, Amyloid, Neurodegenerative disease, Protein aggregation, SOD1",
author = "Yoshiaki Furukawa and Kumi Kaneko and Shoji Watanabe and Koji Yamanaka and Nobuyuki Nukina",
note = "Funding Information: We are grateful to Dr. Gen Matsumoto for the construction of a Neuro2a/FRT cell line. This work was supported by Grants-in-Aid 24111542 (to Y.F.), 23111006 (to K.Y.), 22110004 (to N.N.) for Scientific Research on Innovative Areas , 22240037 for Scientific Research (A) (to N.N.), 25291028 for Scientific Research (B) (to Y.F.), and 24657093 for Challenging Exploratory Research (to Y.F.) from the Ministry of Education, Culture, Sports, Science and Technology of Japan , Grants-in-aid from the Research Committee of CNS Degenerative Diseases , the Ministry of Health, Labour, and Welfare of Japan (to K.Y.), and CREST from Japan Science and Technology Agency (to N.N.).",
year = "2013",
month = aug,
day = "19",
doi = "10.1016/j.febslet.2013.06.046",
language = "English",
volume = "587",
pages = "2500--2505",
journal = "FEBS Letters",
issn = "0014-5793",
publisher = "Elsevier",
number = "16",
}