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FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding

Ashley, Claude T., Wilkinson, Keith D., Reines, Daniel, Warren, Stephen T.·1993·Science

Abstract

Fragile X syndrome is the result of transcriptional suppression of the gene FMR1 as a result of a trinucleotide repeat expansion mutation. The normal function of the FMR1 protein (FMRP) and the mechanism by which its absence leads to mental retardation are unknown. Ribonucleoprotein particle (RNP) domains were identified within FMRP, and RNA was shown to bind in stoichiometric ratios, which suggests that there are two RNA binding sites per FMRP molecule. FMRP was able to bind to its own message with high affinity (dissociation constant = 5.7 nM) and interacted with approximately 4 percent of human fetal brain messages. The absence of the normal interaction of FMRP with a subset of RNA molecules might result in the pleiotropic phenotype associated with fragile X syndrome.

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Metadata

Authors
Ashley, C. T., Wilkinson, K. D., Reines, D., & Warren, S. T.
Venue
Science
Published
1993-10-22
Volume
262
Issue
5133
Pages
563-566
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2036338927
Cited by
741
References
4
FWCI
18.61
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding. https://app.cochat.ai/papers/10.1126/science.7692601
The paper
Ashley, C. T., Wilkinson, K. D., Reines, D., & Warren, S. T. (1993). FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding. Science. https://doi.org/10.1126/science.7692601

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.