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Citation
Ishizuka, A., Siomi, M.C., Siomi, H. (2002). A Drosophila fragile X protein interacts with components of RNAi and ribosomal proteins.  Genes Dev. 16(19): 2497--2508.
FlyBase ID
FBrf0152006
Publication Type
Research paper
Abstract

Fragile X syndrome is a common form of inherited mental retardation caused by the loss of FMR1 expression. The FMR1 gene encodes an RNA-binding protein that associates with translating ribosomes and acts as a negative translational regulator. In Drosophila, the fly homolog of the FMR1 protein (dFMR1) binds to and represses the translation of an mRNA encoding of the microtuble-associated protein Futsch. We have isolated a dFMR1-associated complex that includes two ribosomal proteins, L5 and L11, along with 5S RNA. The dFMR1 complex also contains Argonaute2 (AGO2) and a Drosophila homolog of p68 RNA helicase (Dmp68). AGO2 is an essential component for the RNA-induced silencing complex (RISC), a sequence-specific nuclease complex that mediates RNA interference (RNAi) in Drosophila. We show that Dmp68 is also required for efficient RNAi. We further show that dFMR1 is associated with Dicer, another essential component of the RNAi pathway, and microRNAs (miRNAs) in vivo, suggesting that dFMR1 is part of the RNAi-related apparatus. Our findings suggest a model in which the RNAi and dFMR1-mediated translational control pathways intersect in Drosophila. Our findings also raise the possibility that defects in an RNAi-related machinery may cause human disease.

PubMed ID
PubMed Central ID
PMC187455 (PMC) (EuropePMC)
Related Publication(s)
Review

RNAi and Fragile X.
Anonymous, 2002, Science 298(5593): 497 [FBrf0155370]

Note

A new regulatory pathway for fragile X syndrome?
Hansen and Laird, 2002, Nat. Med. 8(11): 1204--1205 [FBrf0156005]

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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Genes Dev.
    Title
    Genes & Development
    Publication Year
    1987-
    ISBN/ISSN
    0890-9369
    Data From Reference