A Novel Function for Fragile X Mental Retardation Protein in Translational Activation

• DOI: 10.1371/journal.pbio.1000016
• Publication Date: 2009-01-16T11:33:51Z
• Authors: Elias G Bechara; Marie Cecile Didiot; Mireille Melko; Laetitia Davidovic; Mounia Bensaid; Patrick Martin; Marie Castets; Philippe Pognonec; Edouard W Khandjian; Hervé Moine; Barbara Bardoni
• Keywords: Fragile X Syndrome; Posttranslational modification

Fragile X syndrome, the most frequent form of inherited mental retardation, is due to absence Mental Retardation Protein (FMRP), an RNA-binding protein involved in several steps RNA metabolism. To date, two motifs have been found mediate FMRP/RNA interaction, G-quartet and "kissing complex," which both induce translational repression presence FMRP. We show here a new role for FMRP as positive modulator translation. specifically binds Superoxide Dismutase 1 (Sod1) mRNA with high affinity through novel motif, SoSLIP (Sod1 Stem Loops Interacting FMRP), folded three independent stem-loop structures. induces structural modification motif upon its interaction it. also behaves activator whose action potentiated by The results decreased expression Sod1. Because it has observed that brain metabolism FMR1 null mice more sensitive oxidative stress, we propose deregulation Sod1 may be at basis traits physiopathology such anxiety, sleep troubles, autism.