the ras antagonist, farnesylthiosalicylic acid (fts), decreases fibrosis and improves muscle strength in dy2jdy2j mouse model of muscular dystrophyras拮抗剂,farnesylthiosalicylic酸(fts),减少纤维化和改善肌肉力量在dy2jdy2j肌肉萎缩症的小鼠模型.pdfVIP
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the ras antagonist, farnesylthiosalicylic acid (fts), decreases fibrosis and improves muscle strength in dy2jdy2j mouse model of muscular dystrophyras拮抗剂,farnesylthiosalicylic酸(fts),减少纤维化和改善肌肉力量在dy2jdy2j肌肉萎缩症的小鼠模型
The Ras Antagonist, Farnesylthiosalicylic Acid (FTS),
Decreases Fibrosis and Improves Muscle Strength in
dy2J/dy 2J Mouse Model of Muscular Dystrophy
1 . 1. 1 1 1 1
Yoram Nevo * , Shlomit Aga-Mizrachi , Edva Elmakayes , Nurit Yanay , Keren Ettinger , Moran Elbaz ,
1 1 2 2 2 3
Zivia Brunschwig , Oshrat Dadush , Galit Elad-Sfadia , Roni Haklai , Yoel Kloog , Joab Chapman ,
Shimon Reif4
1 Pediatric Neuromuscular Laboratory and the Neuropediatric Unit, Hadassah Hebrew University Hospital, Jerusalem, Israel, 2 Department of Neurobiochemistry, The
George S. Wise Faculty of Life Sciences, Tel-Aviv University, Tel-Aviv, Israel, 3 Department of Neurology, Sheba Medical Center, Tel-Aviv University, Tel-Aviv, Israel,
4 Pediatric Department, Dana Children’s Hospital, Tel-Aviv Sourasky Medical Center, Tel-Aviv University, Tel-Aviv, Israel
Abstract
The Ras superfamily of guanosine-triphosphate (GTP)-binding proteins regulates a diverse spectrum of intracellular
processes involved in inflammation and fibrosis. Farnesythiosalicylic acid (FTS) is a unique and potent Ras inhibitor which
decreased inflammation and fibrosis in experimentally induced liver cirrhosis and ameliorated inflammatory processes in
systemic lupus erythematosus, neuritis and nephritis animal models. FTS effect on Ras expression and activity, muscle
strength and fibrosis was evaluated in the dy2J/dy2J mouse model of merosin deficient congenital muscular dystrophy. The
dy2J/dy2J mice had significantly increased RAS expression and activity compared with the w
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