Browsing by Subject "Lon protease"

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    • Dysfunction of mitochondrial Lon protease and identification of oxidized protein in mouse brain following ... 
      Bulteau, Anne Laure; Mena, Natalia P.; Auchere, Francoise; Lee, Irene; Prigent, Annick; Lobsiger, Christian S.; Camadro, Jean Michel; Hirsch, Etienne C. (Elsevier, 2017)
      Compelling evidence suggests that mitochondrial dysfunction leading to reactive oxygen species (ROS) production and protein oxidation could represent a critical event in the pathogenesis of Parkinson's disease (PD). ...
    • Mutations in the heat-shock protein A9 (HSPA9) gene cause the EVEN-PLUS syndrome of congenital malformations ... 
      Royer Bertrand, Beryl; Castillo Taucher, Silvia; Moreno Salinas, Rodrigo; Cho, Tae-Joon; Chae, Jong-Hee; Choi, Murim; Kim, Ok-Hwa; Dikoglu, Esra; Campos Xavier, Belinda; Girardi, Enrico; Superti Furga, Giulio; Bonafe, Luis; Rivolta, Carlo; Unger, Sheila; Superti Furga, Andrea (Nature Publishing, 2015)
      We and others have reported mutations in LONP1, a gene coding for a mitochondrial chaperone and protease, as the cause of the human CODAS (cerebral, ocular, dental, auricular and skeletal) syndrome (MIM 600373). Here, we ...