TAILIEUCHUNG - Báo cáo khoa học: On the aggregation properties of FMRP – a link with the FXTAS syndrome?

Fragile X mental retardation protein (FMRP) is an RNA binding protein necessary for correct spatiotemporal control of neuronal gene expression in humans. Lack of functional FMRP causes fragile X mental retardation, which is the most common inherited neurodevelopmental disorder in humans. | IFEBS Journal On the aggregation properties of FMRP - a link with the FXTAS syndrome Ljiljana Sjekloca Kris Pauwels and Annalisa Pastore MRC National institute for MedicalResearch London UK Keywords aggregation fragile X mental retardation syndrome fragile X related tremor ataxia syndrome FXTAS protein misfolding structure Correspondence A. Pastore MRC National institute for MedicalResearch The Ridgeway London NW7 1AA UK Fax 44 20 8905 4477 Tel 44 20 8816 2630 E-mail apastor@ Present address Department of Celland Molecular Biology Karolinska Institutet Stockholm SE-171 77 Sweden Received 2 February 2011 revised 20 March 2011 accepted 25 March 2011 doi Fragile X mental retardation protein FMRP is an RNA binding protein necessary for correct spatiotemporal control of neuronal gene expression in humans. Lack of functional FMRP causes fragile X mental retardation which is the most common inherited neurodevelopmental disorder in humans. In a previous study we described the biochemical and biophysical aggregation properties of constructs spanning the conserved region of FMRP and of two other human fragile X related FXR proteins FXR1P and FXR2P. Here we show that the same regions have an intrinsic tendency to aggregate and spontaneously misfold towards b-rich structures also under non-destabilizing conditions. These findings pave the way to future studies of the mechanism of formation of FXR-containing ribonucleoprotein granules and suggest a possible link with the as yet poorly understood FXR proteins associated pathologies. Structured digital abstract FXR2P binds to FXR2P by fluorescence technology View interaction FMRP binds to FMRP by electron microscopy View interaction FXR1P binds to FXR1P by electron microscopy View interaction Introduction The fragile X mental retardation protein FMRP is an 70 kDa human protein encoded by the X-linked gene FMR1 which is expressed in different organs most prominently in brain and .

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