The DOI matches its Crossref record; 54 of the 54 references it cites were found in a registry or an index.
Trapping of messenger RNA by Fragile X Mental Retardation protein into cytoplasmic granules induces translation repression
Abstract
Absence of Fragile X Mental Retardation Protein (FMRP), an RNA-binding protein, is responsible for the Fragile X syndrome, the most common form of inherited mental retardation. FMRP is a cytoplasmic protein associated with mRNP complexes containing poly(A)+mRNA. As a step towards understanding FMRP function(s), we have established the immortal STEK Fmr1 KO cell line and showed by transfection assays with FMR1-expressing vectors that newly synthesized FMRP accumulates into cytoplasmic granules. These structures contain mRNAs and several other RNA-binding proteins. The formation of these cytoplasmic granules is dependent on determinants located in the RGG domain. We also provide evidence that FMRP acts as a translation repressor following co-transfection with reporter genes. The FMRP-containing mRNPs are dynamic structures that oscillate between polyribosomes and cytoplasmic granules reminiscent of the Stress Granules that contain repressed mRNAs. We speculate that, in neurons, FMRP plays a role as a mRNA repressor in incompetent mRNP granules that have to be translocated from the cell body to distal locations such as dendritic spines and synaptosomes.
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Cites
hnRNP PROTEINS AND THE BIOGENESIS OF mRNA
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Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
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Fmr1 knockout mice: A model to study fragile X mental retardation
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A Requirement for Local Protein Synthesis in Neurotrophin-Induced Hippocampal Synaptic Plasticity
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Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function
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Dynamic Shuttling of Tia-1 Accompanies the Recruitment of mRNA to Mammalian Stress Granules
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Weiler, I. J., Irwin, S. A., Klintsova, A. Y., Spencer, C. M., Brazelton, A. D., Miyashiro, K., Comery, T. A., Patel, B., Eberwine, J., & Greenough, W. T. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.10.5395 · Cited by 644 · cited 2×
“(12) have shown that Fmr1 mRNA is present in isolated synaptosomes purified from rat brain and that this mRNA is rapidly taken up into polyribosomes in response to stimulation by agonists for phosphoinositide-coupled metabotropic glutamate receptors.”
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Cited by
Fragile X Syndrome: Loss of Local mRNA Regulation Alters Synaptic Development and Function
Bassell, G. J., & Warren, S. T. (2008) · Neuron · doi:10.1016/j.neuron.2008.10.004 · Cited by 1,051
Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., & Bagni, C. (2003) · Cell · doi:10.1016/s0092-8674(03)00079-5 · Cited by 676
AU-rich-element-mediated upregulation of translation by FXR1 and Argonaute 2
Vasudevan, S., & Steitz, J. A. (2007) · Cell · doi:10.1016/j.cell.2007.01.038 · Cited by 656
Molecular Mechanisms of Fragile X Syndrome: A Twenty-Year Perspective
Santoro, M. R., Bray, S. M., & Warren, S. T. (2012) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev-pathol-011811-132457 · Cited by 528
Osterweil, E. K., Krueger, D. D., Reinhold, K., & Bear, M. F. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.3888-10.2010 · Cited by 384
Metadata
- Authors
- Mazroui, R.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2002-11-15
- Volume
- 11
- Issue
- 24
- Pages
- 3007-3017
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2152703066
- Cited by
- 369
- References
- 54
- FWCI
- 8.17
- Type
- journal article
- Language
- en
- Open access
- closed
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