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Nat Commun ; 10(1): 1710, 2019 04 12.
Artigo em Inglês | MEDLINE | ID: mdl-30979884

RESUMO

Whether fragile X mental retardation protein (FMRP) target mRNAs and neuronal activity contributing to elevated basal neuronal protein synthesis in fragile X syndrome (FXS) is unclear. Our proteomic experiments reveal that the de novo translational profile in FXS model mice is altered at steady state and in response to metabotropic glutamate receptor (mGluR) stimulation, but the proteins expressed differ under these conditions. Several altered proteins, including Hexokinase 1 and Ras, also are expressed in the blood of FXS model mice and pharmacological treatments previously reported to ameliorate phenotypes modify their abundance in blood. In addition, plasma levels of Hexokinase 1 and Ras differ between FXS patients and healthy volunteers. Our data suggest that brain-based de novo proteomics in FXS model mice can be used to find altered expression of proteins in blood that could serve as disease-state biomarkers in individuals with FXS.


Assuntos
Síndrome do Cromossomo X Frágil/metabolismo , Receptores de Glutamato Metabotrópico/metabolismo , Adolescente , Adulto , Animais , Biomarcadores/sangue , Modelos Animais de Doenças , Feminino , Síndrome do Cromossomo X Frágil/genética , Hexoquinase/sangue , Hipocampo/metabolismo , Humanos , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Fenótipo , Adulto Jovem , Proteínas ras/metabolismo
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