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Citation
Cvetkovska, V., Hibbert, A.D., Emran, F., Chen, B.E. (2013). Overexpression of Down syndrome cell adhesion molecule impairs precise synaptic targeting.  Nat. Neurosci. 16(6): 677--682.
FlyBase ID
FBrf0221678
Publication Type
Research paper
Abstract

Fragile X syndrome is caused by the loss of Fragile X mental retardation protein (FMRP), an RNA-binding protein that suppresses protein translation. We found that FMRP binds to Down syndrome cell adhesion molecule (Dscam) RNA, a molecule that is involved in neural development and has been implicated in Down syndrome. Elevated Dscam protein levels in FMRP null Drosophila and in flies with three copies of the Dscam gene both produced specific and similar synaptic targeting errors in a hard-wired neural circuit, which impaired the flies' sensory perception. Reducing Dscam levels in FMRP null flies reduced synaptic targeting errors and rescued behavioral responses. Our results indicate that excess Dscam protein may be a common molecular mechanism underlying altered neural wiring in intellectual disabilities such as Fragile X and Down syndromes.

PubMed ID
PubMed Central ID
PMC3954815 (PMC) (EuropePMC)
Related Publication(s)
Note

Neurodevelopmental disorders: when more is less?
Flight, 2013, Nat. Rev. Neurosci. 14(7): 458--459 [FBrf0222543]

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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Nat. Neurosci.
    Title
    Nature Neuroscience
    Publication Year
    1998-
    ISBN/ISSN
    1097-6256
    Data From Reference
    Aberrations (1)
    Alleles (12)
    Genes (4)
    Human Disease Models (3)
    Physical Interactions (4)
    Natural transposons (1)
    Insertions (1)
    Experimental Tools (1)
    Transgenic Constructs (4)