Developmental expression of the neuroligins and neurexins in fragile X mice

2015 ◽  
Vol 524 (4) ◽  
pp. 807-828 ◽  
Author(s):  
Jonathan K.Y. Lai ◽  
Laurie C. Doering ◽  
Jane A. Foster
Keyword(s):  
Fragile X ◽  
Developmental Expression

scholarly journals Astroglial FMRP deficiency cell-autonomously up-regulates miR-128 and disrupts developmental astroglial mGluR5 signaling

2020 ◽  
Vol 117 (40) ◽  
pp. 25092-25103
Author(s):  
Yuqin Men ◽  
Liang Ye ◽  
Ryan D. Risgaard ◽  
Vanessa Promes ◽  
Xinyu Zhao ◽  
...  
Keyword(s):  
Protein Expression ◽  
Molecular Mechanisms ◽  
Metabotropic Glutamate Receptor ◽  
Fragile X ◽  
Developmental Expression ◽  
Metabotropic Glutamate Receptor 5 ◽  
Metabotropic Glutamate ◽  
Mental Retardation Protein ◽  
Dependent Cell

The loss of fragile X mental retardation protein (FMRP) causes fragile X syndrome (FXS), the most common inherited intellectual disability. How the loss of FMRP alters protein expression and astroglial functions remains essentially unknown. Here we showed that selective loss of astroglial FMRP in vivo up-regulates a brain-enriched miRNA, miR-128-3p, in mouse and human FMRP-deficient astroglia, which suppresses developmental expression of astroglial metabotropic glutamate receptor 5 (mGluR5), a major receptor in mediating developmental astroglia to neuron communication. Selective in vivo inhibition of miR-128-3p in FMRP-deficient astroglia sufficiently rescues decreased mGluR5 function, while astroglial overexpression of miR-128-3p strongly and selectively diminishes developmental astroglial mGluR5 signaling. Subsequent transcriptome and proteome profiling further suggests that FMRP commonly and preferentially regulates protein expression through posttranscriptional, but not transcriptional, mechanisms in astroglia. Overall, our study defines an FMRP-dependent cell-autonomous miR pathway that selectively alters developmental astroglial mGluR5 signaling, unveiling astroglial molecular mechanisms involved in FXS pathogenesis.

Developmental expression of FMRP in the astrocyte lineage: Implications for fragile X syndrome

Glia ◽  
2007 ◽  
Vol 55 (15) ◽  
pp. 1601-1609 ◽  
Author(s):  
Laura K. K. Pacey ◽  
Laurie C. Doering
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Developmental Expression

Axonal localization of the Fragile X family of RNA binding proteins is conserved across mammals

2018 ◽  
Author(s):  
Katherine A. Shepard ◽  
Lulu I T. Korsak ◽  
Michael R. Akins
Keyword(s):  
Binding Proteins ◽  
Rna Binding ◽  
Rna Binding Proteins ◽  
Fragile X ◽  
Mossy Fibers ◽  
Developmental Expression ◽  
Divergent Evolution ◽  
Mammalian Brain ◽  
Local Translation ◽  
Related Family

AbstractSpatial segregation of proteins to neuronal axons arises in part from local translation of select mRNAs that are first transported into axons in ribonucleoprotein particles (RNPs), complexes containing mRNAs and RNA binding proteins. Understanding the importance of local translation for a particular circuit requires not only identifying axonal RNPs and their mRNA cargoes, but also whether these RNPs are broadly conserved or restricted to only a few species. Fragile X granules (FXGs) are axonal RNPs containing the Fragile X related family of RNA binding proteins along with ribosomes and specific mRNAs. FXGs were previously identified in mouse, rat, and human brains in a conserved subset of neuronal circuits but with species-dependent developmental profiles. Here we asked whether FXGs are a broadly conserved feature of the mammalian brain and sought to better understand the species-dependent developmental expression pattern. We found FXGs in a conserved subset of neurons and circuits in the brains of every examined species that together include mammalian taxa separated by up to 160 million years of divergent evolution. A developmental analysis of rodents revealed that FXG expression in frontal cortex and olfactory bulb followed consistent patterns in all species examined. In contrast, FXGs in hippocampal mossy fibers showed an increase in abundance across development for most species except for guinea pigs and members of the Mus genus, animals that navigate particularly small home ranges in the wild. The widespread conservation of FXGs suggests that axonal translation is an ancient, conserved mechanism for regulating the proteome of mammalian axons.

scholarly journals Developmental expression of Xenopus Fragile X mental retardation-1 gene

2005 ◽  
Vol 49 (8) ◽  
pp. 981-984 ◽  
Author(s):  
Jae H. Lim ◽  
Ting Luo ◽  
Thomas D. Sargent ◽  
Justin R. Fallon
Keyword(s):  
Mental Retardation ◽  
Fragile X ◽  
Developmental Expression ◽  
Fragile X Mental Retardation
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