The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses.

Détails

ID Serval
serval:BIB_4575DBFBB309
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses.
Périodique
Cell
Auteur(s)
Zalfa F., Giorgi M., Primerano B., Moro A., Di Penta A., Reis S., Oostra B., Bagni C.
ISSN
0092-8674 (Print)
ISSN-L
0092-8674
Statut éditorial
Publié
Date de publication
07/02/2003
Volume
112
Numéro
3
Pages
317-327
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, Non-P.H.S. ; Research Support, U.S. Gov't, P.H.S.
Publication Status: ppublish
Résumé
The Fragile X syndrome, which results from the absence of functional FMRP protein, is the most common heritable form of mental retardation. Here, we show that FMRP acts as a translational repressor of specific mRNAs at synapses. Interestingly, FMRP associates not only with these target mRNAs, but also with the dendritic, non-translatable RNA BC1. Blocking of BC1 inhibits the interaction of FMRP with its target mRNAs. Furthermore, BC1 binds directly to FMRP and can also associate, in the absence of any protein, with the mRNAs regulated by FMRP. This suggests a mechanism where BC1 could determine the specificity of FMRP function by linking the regulated mRNAs and FMRP. Thus, when FMRP is not present, loss of translational repression of specific mRNAs at synapses could result in synaptic dysfunction phenotype of Fragile X patients.

Mots-clé
Animals, Base Sequence/genetics, Brain/growth & development, Brain/metabolism, Dendrites/metabolism, Fragile X Mental Retardation Protein, Fragile X Syndrome/genetics, Fragile X Syndrome/metabolism, Genes, Suppressor/physiology, Humans, Macromolecular Substances, Mice, Mice, Knockout, Molecular Sequence Data, Molecular Structure, Nerve Tissue Proteins/deficiency, Nerve Tissue Proteins/genetics, Protein Binding/genetics, Protein Biosynthesis/genetics, RNA, Messenger/genetics, RNA, Messenger/metabolism, RNA, Small Cytoplasmic/genetics, RNA, Small Cytoplasmic/metabolism, RNA-Binding Proteins, Ribonucleoproteins/genetics, Ribonucleoproteins/metabolism, Synapses/genetics, Synapses/metabolism, Synaptic Transmission/genetics, Tumor Cells, Cultured
Pubmed
Création de la notice
06/03/2017 18:23
Dernière modification de la notice
03/03/2018 16:45
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