The DOI matches its Crossref record; 55 of the 55 references it cites were found in a registry or an index.
The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals
Abstract
Fragile X syndrome is a frequent cause of mental retardation resulting from the absence of FMRP, the protein encoded by the FMR1 gene. FMRP is an RNA-binding protein of unknown function which is associated with ribosomes. To gain insight into FMRP function, we performed immunolocalization analysis of FMRP truncation and fusion constructs which revealed a nuclear localization signal (NLS) in the amino terminus of FMRP as well as a nuclear export signal (NES) encoded by exon 14. A 17 amino acid peptide containing the FMRP NES, which closely resembles the NES motifs recently described for HIV-1 Rev and PKI, is sufficient to direct nuclear export of a microinjected protein conjugate. Sucrose gradient analysis shows that FMRP ribosome association is RNA-dependent and FMRP is found in ribonucleoprotein (RNP) particles following EDTA treatment. These data are consistent with nascent FMRP entering the nucleus to assemble into mRNP particles prior to export back into the cytoplasm and suggests that fragile X syndrome may result from altered translation of transcripts which normally bind to FMRP.
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“FMRP is an RNA-binding protein, and, despite its clear shuttling from the nucleus to the cytoplasm (59,60), only the cytoplasmic function of FMRP has been well characterized.”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [59]
Citations
Cites
Verkerk, A. J. M. H., Pieretti, M., Sutcliffe, J. S., Fu, Y.-H., Kuhl, D. P. A., Pizzuti, A., Reiner, O., Richards, S., Victoria, M. F., Zhang, F., et al. (1991) · Cell · doi:10.1016/0092-8674(91)90397-h · Cited by 3,481
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Instability of a 550-Base Pair DNA Segment and Abnormal Methylation in Fragile X Syndrome
Oberlé, I., Rousseau, F., Heitz, D., Kretz, C., Devys, D., Hanauer, A., Boué, J., Bertheas, M. F., & Mandel, J. L. (1991) · Science · doi:10.1126/science.252.5009.1097 · Cited by 1,499
Identification of a signal for rapid export of proteins from the nucleus
Wen, W., Meinkotht, J. L., Tsien, R. Y., & Taylor, S. S. (1995) · Cell · doi:10.1016/0092-8674(95)90435-2 · Cited by 1,190
Fischer, U., Huber, J., Boelens, W. C., Mattaj, I. W., & Lührmann, R. (1995) · Cell · doi:10.1016/0092-8674(95)90436-0 · Cited by 1,140
Görlich, D., & Mattaj, I. W. (1996) · Science · doi:10.1126/science.271.5255.1513 · Cited by 1,081
Devys, D., Lutz, Y., Rouyer, N., Bellocq, J., & Mandel, J. (1993) · Nature Genetics · doi:10.1038/ng0893-335 · Cited by 781
FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding
Ashley, C. T., Wilkinson, K. D., Reines, D., & Warren, S. T. (1993) · Science · doi:10.1126/science.7692601 · Cited by 752
DNA methylation represses FMR-1 transcription in fragile X syndrome
Sutcliffe, J. S., Nelson, D. L., Zhang, F., Pieretti, M., Caskey, C. T., Saxe, D., & Warren, S. T. (1992) · Human Molecular Genetics · doi:10.1093/hmg/1.6.397 · Cited by 680
The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein.
Siomi, H., Siomi, M. C., Nussbaum, R. L., & Dreyfuss, G. (1993) · Cell · doi:10.1016/0092-8674(93)90420-u · Cited by 641
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Brown, V., Jin, P., Ceman, S., Darnell, J. C., O'Donnell, W. T., Tenenbaum, S. A., Jin, X., Feng, Y., Wilkinson, K. D., Keene, J. D., et al. (2001) · Cell · doi:10.1016/s0092-8674(01)00568-2 · Cited by 1,109
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
Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function
Darnell, J. C., Jensen, K. B., Jin, P., Brown, V., Warren, S. T., & Darnell, R. B. (2001) · Cell · doi:10.1016/s0092-8674(01)00566-9 · Cited by 952
Serotonin receptor 1A knockout: An animal model of anxiety-related disorder
Ramboz, S., Oosting, R. S., Amara, D. A., Kung, H. F., Blier, P., Mendelsohn, M. M., Mann, J. J., Brunner, D., & Hen, R. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.24.14476 · Cited by 831
FMR1 and the fragile X syndrome: Human genome epidemiology review
Crawford, D. C., Acuña, J. M., & Sherman, S. L. (2001) · Genetics in Medicine · doi:10.1097/00125817-200109000-00006 · Cited by 711
Metadata
- Authors
- Eberhart, D.
- Affiliations
- Howard Hughes Medical Institute (US)
- Corresponding author
- Eberhart, D.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 1996-08-01
- Volume
- 5
- Issue
- 8
- Pages
- 1083-1091
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2106239245
- Cited by
- 390
- References
- 53
- Citations by year
- 2026: 3 · 2025: 7 · 2024: 3 · 2023: 4 · 2022: 6 · 2021: 7 · 2020: 7 · 2019: 11 · 2018: 13 · 2017: 10 · 2016: 11 · 2015: 13 · 2014: 17 · 2013: 21 · 2012: 21
- FWCI
- 9.58
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Human Molecular Genetics (publishedVersion)
- PubMed (publishedVersion)
- HighWire Press Open Archive (submittedVersion)
- Funders
- Howard Hughes Medical Institute
- National Institutes of Health
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD 20521)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA Research and Splicing
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Keywords
- fragile X syndrome, FMRP, FMR1 gene, nuclear localization signal, nuclear export signal, ribonucleoprotein, RNA-binding proteins, ribosome association, exon 14, protein localization, translational regulation
- MeSH terms
- Amino Acid Sequence, Animals, Biological Transport, Active, Cell Nucleus, Exons, Fragile X Syndrome, Humans, Models, Biological, Molecular Sequence Data, Nerve Tissue Proteins, Recombinant Fusion Proteins, Ribonucleoproteins, Ribosomes, Transfection, RNA-Binding Proteins, COS Cells, Protein Sorting Signals, Fragile X Messenger Ribonucleoprotein 1
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