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Highlights • ATP synthase c-subunit leak in Fragile X causes aberrant metabolism • Changes in ATP synthase component stoichiometry regulate protein synthesis rate • Inhibition of the leak normalizes synaptic spine morphology and Fragile X behavior Summary Loss of the gene (Fmr1) encoding Fragile X mental retardation protein (FMRP) causes increased mRNA translation and aberrant synaptic development.
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