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Ljiljana Sjekloća, Kris Pauwels and Annalisa Pastore On the aggregation properties of FMRP – a link with the FXTAS syndrome? The FEBS Journal 278

Article first published online: 9 MAY 2011 | DOI: 10.1111/j.1742-4658.2011.08108.x

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Lack of the Fragile X mental retardation protein (FMRP) causes an inherited mental retardation in humans. We describe here that constructs spanning the conserved N-terminus of FMRP and of the two homologues, FXR1P and FXR2P, have an intrinsic tendency to aggregate and misfold towards beta-rich structures, also under non-destabilizing conditions. These findings suggest a link with FMRP associated pathologies.

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