Cited by 677Genetics and Neurodevelopmental DisordersRNA Research and SplicingRNA and protein synthesis mechanisms

The DOI matches its Crossref record; 63 of the 65 references it cites were found in a registry or an index.

The Fragile X Syndrome Protein FMRP Associates with BC1 RNA and Regulates the Translation of Specific mRNAs at Synapses

Open access

Zalfa, Francesca, Giorgi, Marcello, Primerano, Beatrice, Moro, Annamaria, Di Penta, Alessandra, Reis, Surya, Oostra, Ben, Bagni, Claudia·2003·Cell

DOI 10.1016/s0092-8674(03)00079-5

Open this paper in CoChat — free account Open PDF

Abstract

The Fragile X syndrome, which results from the absence of functional FMRP protein, is the most common heritable form of mental retardation. Here, we show that FMRP acts as a translational repressor of specific mRNAs at synapses. Interestingly, FMRP associates not only with these target mRNAs, but also with the dendritic, non-translatable RNA BC1. Blocking of BC1 inhibits the interaction of FMRP with its target mRNAs. Furthermore, BC1 binds directly to FMRP and can also associate, in the absence of any protein, with the mRNAs regulated by FMRP. This suggests a mechanism where BC1 could determine the specificity of FMRP function by linking the regulated mRNAs and FMRP. Thus, when FMRP is not present, loss of translational repression of specific mRNAs at synapses could result in synaptic dysfunction phenotype of Fragile X patients.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Integrity signals

Registry

Crossref · 10.1016/s0092-8674(03)00079-5

Cites

63 of 63 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome
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
Self-citation
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
Arc, a growth factor and activity-regulated gene, encodes a novel cytoskeleton-associated protein that is enriched in neuronal dendrites
Lyford, G. L., Yamagata, K., Kaufmann, W. E., Barnes, C. A., Sanders, L. K., Copeland, N. G., Gilbert, D. J., Jenkins, N. A., Lanahan, A. A., & Worley, P. F. (1995) · Neuron · doi:10.1016/0896-6273(95)90299-6 · Cited by 1,212
Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome
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
Fmr1 knockout mice: A model to study fragile X mental retardation
THEDUTCHBELGIANFRAGILEXCONSOR (1994) · Cell · doi:10.1016/0092-8674(94)90569-x · Cited by 1,108
Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
Comery, T. A., Harris, J. B., Willems, P. J., Oostra, B. A., Irwin, S. A., Weiler, I. J., & Greenough, W. T. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.10.5401 · Cited by 1,046
Self-citation
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
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
Protein synthesis at synaptic sites on dendrites.
Steward, O., & Schuman, E. (2001) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.24.1.299 · Cited by 731
Somatodendritic expression of an immediate early gene is regulated by synaptic activity.
Link, W., Konietzko, U., Kauselmann, G., Krug, M., Schwanke, B., Frey, U., & Kuhl, D. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.12.5734 · Cited by 710

Show all 65 references

Research works printed but not linked: 2

Bakker, 1994, FMR1 knockout mice, Cell, 78, 23
Hirst, 1995, Two new cases of FMR1 deletion associated with mental impairment, Am. J. Hum. Genet., 56, 67

Sorted by citations

Items 301–400 of 677

PRINS, a primate-specific long non-coding RNA, plays a role in the keratinocyte stress response and psoriasis pathogenesis
Széll, M., Danis, J., Bata‐Csörgõ, Z., & Kemény, L. V. (2016) · Pflügers Archiv - European Journal of Physiology · doi:10.1007/s00424-016-1803-z · Cited by 55
Translational Control at the Synapse
Wang, H., & Tiedge, H. (2004) · The Neuroscientist · doi:10.1177/1073858404265866 · Cited by 55
RNA localisation in the nervous system
Dahm, R., Kiebler, M. A., & Macchi, P. (2007) · Seminars in Cell and Developmental Biology · doi:10.1016/j.semcdb.2007.01.009 · Cited by 51
Expression of BC1 Impairs Spatial Learning and Memory in Alzheimer’s Disease Via APP Translation
Zhang, T., Pang, P., Fang, Z., Guo, Y., Li, H., Li, X., Tian, T., Yang, X., Chen, W., Shu, S., et al. (2018) · Molecular Neurobiology · doi:10.1007/s12035-017-0820-z · Cited by 52
Sertraline May Improve Language Developmental Trajectory in Young Children with Fragile X Syndrome: A Retrospective Chart Review
Indah Winarni, T., Chonchaiya, W., Adams, E., Au, J., Mu, Y., Rivera, S. M., Nguyen, D. V., & Hagerman, R. J. (2012) · Autism Research and Treatment · doi:10.1155/2012/104317 · Cited by 52
Dendritic mRNA Targeting and Translation
Kindler, S., & Kreienkamp, H. (2012) · Advances in experimental medicine and biology · doi:10.1007/978-3-7091-0932-8_13 · Cited by 54
Whole genome microarray analysis of gene expression in subjects with fragile X syndrome
Bittel, D. C., Kibiryeva, N., & Butler, M. G. (2007) · Genetics in Medicine · doi:10.1097/gim.0b013e3180ca9a9a · Cited by 52
Pharmacological Rescue of Cortical Synaptic and Network Potentiation in a Mouse Model for Fragile X Syndrome
Chen, T., Lu, J.-S., Song, Q., Liu, M.-G., Koga, K., Descalzi, G., Li, Y.-Q., & Zhuo, M. (2014) · Neuropsychopharmacology · doi:10.1038/npp.2014.44 · Cited by 52
In vivo neuronal function of the fragile X mental retardation protein is regulated by phosphorylation
Coffee, R. L., Williamson, A. J., Adkins, C. M., Gray, M. C., Page, T. L., & Broadie, K. S. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr527 · Cited by 51
Elevated CaMKIIα and Hyperphosphorylation of Homer Mediate Circuit Dysfunction in a Fragile X Syndrome Mouse Model
Guo, W., Ceolin, L., Collins, K. A., Perroy, J., & Huber, K. M. (2015) · Cell Reports · doi:10.1016/j.celrep.2015.11.013 · Cited by 53
Proteome-Wide Characterization of the RNA-Binding Protein RALY-Interactome Using the in Vivo-Biotinylation-Pulldown-Quant (iBioPQ) Approach
Tenzer, S., Moro, A., Kuharev, J., Francis, A. C., Vidalino, L., Provenzani, A., & Macchi, P. (2013) · Journal of Proteome Research · doi:10.1021/pr400193j · Cited by 52
Self-citation
Absence of the Fragile X Mental Retardation Protein results in defects of RNA editing of neuronal mRNAs in mouse
Filippini, A., Bonini, D., Lacoux, C., Pacini, L., Zingariello, M., Sancillo, L., Bosisio, D., Salvi, V., Mingardi, J., Via, L. L., et al. (2017) · RNA Biology · doi:10.1080/15476286.2017.1338232 · Cited by 56
Self-citation
Interneuron Dysfunction and Inhibitory Deficits in Autism and Fragile X Syndrome
Nomura, T. (2021) · Cells · doi:10.3390/cells10102610 · Cited by 51
Mutant Mouse Models of Autism Spectrum Disorders
Provenzano, G., Zunino, G., Genovesi, S., Sgadó, P., & Bozzi, Y. (2012) · Disease Markers · doi:10.1155/2012/916082 · Cited by 53
Drosophila FMRP regulates microtubule network formation and axonal transport of mitochondria
Yao, A., Jin, S., Li, X., Liu, Z., Ma, X., Tang, J., & Zhang, Y. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq431 · Cited by 50
Disruptive variants of CSDE1 associate with autism and interfere with neuronal development and synaptic transmission
Guo, H., Li, Y., Shen, L., Wang, T., Jia, X., Liu, L., Xu, T., Ou, M., Hoekzema, K., Wu, H., et al. (2019) · Science Advances · doi:10.1126/sciadv.aax2166 · Cited by 49
Temporal requirements of the fragile X mental retardation protein in modulating circadian clock circuit synaptic architecture
Gatto, C. L. (2009) · Frontiers in Neural Circuits · doi:10.3389/neuro.04.008.2009 · Cited by 50
RNA‐binding proteins involved in RNA localization and their implications in neuronal diseases
Tolino, M., Köhrmann, M., & Kiebler, M. A. (2012) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2012.08160.x · Cited by 51
Molecular and Cellular Aspects of Mental Retardation in the Fragile X Syndrome: From Gene Mutation/s to Spine Dysmorphogenesis
De Rubeis, S., Fernández, E., Buzzi, A., Di Marino, D., & Bagni, C. (2012) · Advances in Experimental Medicine and Biology · doi:10.1007/978-3-7091-0932-8_23 · Cited by 48
Self-citation
The synaptic life of microtubules
Waites, C. L., Qu, X., & Bartolini, F. (2021) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2021.03.004 · Cited by 45
Impact of acamprosate on plasma amyloid-β precursor protein in youth: A pilot analysis in fragile X syndrome-associated and idiopathic autism spectrum disorder suggests a pharmacodynamic protein marker
Erickson, C. A., Ray, B., Maloney, B., Wink, L. K., Bowers, K. A., Schaefer, T. L., McDougle, C. J., Sokol, D. K., & Lahiri, D. K. (2014) · Journal of Psychiatric Research · doi:10.1016/j.jpsychires.2014.07.011 · Cited by 54
SINE RNA Induces Severe Developmental Defects in Arabidopsis thaliana and Interacts with HYL1 (DRB1), a Key Member of the DCL1 Complex
Pouch-Pélissier, M.-N., Pélissier, T., Elmayan, T., Vaucheret, H., Boko, D., Jantsch, M. F., & Deragon, J. (2008) · PLoS Genetics · doi:10.1371/journal.pgen.1000096 · Cited by 48
Post-training disruption of Arc protein expression in the anterior cingulate cortex impairs long-term memory for inhibitory avoidance training
Holloway-Erickson, C. M., & McIntyre, C. K. (2011) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2011.02.002 · Cited by 48
The fragile X mental retardation protein regulates tumor invasiveness-related pathways in melanoma cells
Zalfa, F., Panasiti, V., Carotti, S., Zingariello, M., Perrone, G., Sancillo, L., Pacini, L., Luciani, F., Roberti, V., D’Amico, S., et al. (2017) · Cell Death and Disease · doi:10.1038/cddis.2017.521 · Cited by 48
Self-citation
BC200 (BCYRN1) – The shortest, long, non-coding RNA associated with cancer
Samson, J., Cronin, S., & Dean, K. A. (2018) · Non-coding RNA Research · doi:10.1016/j.ncrna.2018.05.003 · Cited by 50
Differential usage of transcriptional start sites and polyadenylation sites in FMR1 premutation alleles
Tassone, F., Rubeis, S. D., Carosi, C., Fata, G. L., Serpa, G., Raske, C., Willemsen, R., Hagerman, P. J., & Bagni, C. (2011) · Nucleic Acids Research · doi:10.1093/nar/gkr100 · Cited by 48
Self-citation
How strong is the case for regulation of the initiation step of translation by elements at the 3′ end of eukaryotic mRNAs?
Kozak, M. (2004) · Gene · doi:10.1016/j.gene.2004.08.011 · Cited by 49
Neuronal PAS domain proteins 1 and 3 are master regulators of neuropsychiatric risk genes
Michaelson, J. J., Shin, M.-K., Koh, J.-Y., Brueggeman, L., Zhang, A., Katzman, A., McDaniel, L., Fang, M., Pufall, M., & Pieper, A. A. (2017) · Biological Psychiatry · doi:10.1016/j.biopsych.2017.03.021 · Cited by 47
Molecular and synaptic defects in intellectual disability syndromes
Verpelli, C., & Sala, C. (2011) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2011.09.007 · Cited by 55
From FMRP Function to Potential Therapies for Fragile X Syndrome
Sethna, F., Moon, C., & Wang, H. (2013) · Neurochemical Research · doi:10.1007/s11064-013-1229-3 · Cited by 49
Fragile X mental retardation protein is a Zika virus restriction factor that is antagonized by subgenomic flaviviral RNA
Soto-Acosta, R., Xie, X., Shan, C., Baker, C. K., Shi, P., Rossi, S. L., García-Blanco, M. A., & Bradrick, S. S. (2018) · eLife · doi:10.7554/elife.39023 · Cited by 48
The epitranscriptome and synaptic plasticity
Flamand, M. N., & Meyer, K. D. (2019) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2019.04.007 · Cited by 47
The FRAXopathies: Definition, overview, and update
Pirozzi, F., Tabolacci, E., & Neri, G. (2011) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.34113 · Cited by 48
Role of CTCF Protein in Regulating FMR1 Locus Transcription
Lanni, S., Goracci, M., Borrelli, L., Mancano, G., Chiurazzi, P., Moscato, U., Ferrè, F., Helmer‐Citterich, M., Tabolacci, E., & Neri, G. (2013) · PLoS Genetics · doi:10.1371/journal.pgen.1003601 · Cited by 46
Long noncoding RNAs in psychiatric disorders
Zuo, L., Tan, Y., Wang, Z., Wang, K.-S., Zhang, X., Chen, X., Li, C. R., Wang, T., & Luο, X. (2016) · Psychiatric Genetics · doi:10.1097/ypg.0000000000000129 · Cited by 49
Reduced Lateral Inhibition Impairs Olfactory Computations and Behaviors in a Drosophila Model of Fragile X Syndrome
Franco, L. M., Okray, Z., Linneweber, G. A., Hassan, B. A., & Yaksi, E. (2017) · Current Biology · doi:10.1016/j.cub.2017.02.065 · Cited by 48
Fragile X-related protein family: a double-edged sword in neurodevelopmental disorders and cancer
Majumder, M., Johnson, R. H., & Palanisamy, V. (2020) · Critical Reviews in Biochemistry and Molecular Biology · doi:10.1080/10409238.2020.1810621 · Cited by 50
The enigmatic world of mRNA-like ncRNAs: Their role in human evolution and in human diseases
Széll, M., Bata‐Csörgõ, Z., & Kemény, L. V. (2008) · Seminars in Cancer Biology · doi:10.1016/j.semcancer.2008.01.007 · Cited by 46
Dendritic protein synthesis in the normal and diseased brain
Swanger, S. A., & Bassell, G. J. (2012) · Neuroscience · doi:10.1016/j.neuroscience.2012.12.003 · Cited by 51
JAKMIP1, a Novel Regulator of Neuronal Translation, Modulates Synaptic Function and Autistic-like Behaviors in Mouse
Berg, J. M., Lee, C., Chen, L., Galvan, L., Cepeda, C., Chen, J. Y., Peñagarikano, O., Stein, J. L., Li, A., Oguro-Ando, A., et al. (2015) · Neuron · doi:10.1016/j.neuron.2015.10.031 · Cited by 45
Dysregulated Translation in Neurodevelopmental Disorders: An Overview of Autism‐Risk Genes Involved in Translation
Chen, Y., Chang, Y., & Huang, Y. (2018) · Developmental Neurobiology · doi:10.1002/dneu.22653 · Cited by 42
The fragile X mental retardation protein developmentally regulates the strength and fidelity of calcium signaling in Drosophila mushroom body neurons
Tessier, C. R., & Broadie, K. S. (2010) · Neurobiology of Disease · doi:10.1016/j.nbd.2010.09.002 · Cited by 44
Defects in translational regulation contributing to human cognitive and behavioral disease
Darnell, J. (2011) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2011.05.002 · Cited by 43
Regulatory BC1 RNA and the Fragile X Mental Retardation Protein: Convergent Functionality in Brain
Zhong, J. W., Chuang, S.-C., Bianchi, R., Zhao, W., Paul, G., Thakkar, P., Liu, D., Fenton, A. A., Wong, R. K. S., & Tiedge, H. (2010) · PLoS ONE · doi:10.1371/journal.pone.0015509 · Cited by 43
The DHX33 RNA Helicase Promotes mRNA Translation Initiation
Zhang, Y., You, J., Wang, X., & Weber, J. D. (2015) · Molecular and Cellular Biology · doi:10.1128/mcb.00315-15 · Cited by 43
Chapter 8 Translational Regulatory Mechanisms in Synaptic Plasticity and Memory Storage
Costa‐Mattioli, M., Sonenberg, N., & Richter, J. D. (2009) · Progress in molecular biology and translational science · doi:10.1016/s1877-1173(09)90008-4 · Cited by 43
The double-edged sword of long non-coding RNA: The role of human brain-specific BC200 RNA in translational control, neurodegenerative diseases, and cancer
Sosińska, P., Mikuła‐Pietrasik, J., & Książek, K. (2015) · Mutation Research/Reviews in Mutation Research · doi:10.1016/j.mrrev.2015.08.002 · Cited by 45
Structural determinants of the SINE B2 element embedded in the long non-coding RNA activator of translation AS Uchl1
Podbevšek, P., Fasolo, F., Bon, C., Cimatti, L., Reißer, S., Carninci, P., Bussi, G., Zucchelli, S., Plavec, J., & Gustincich, S. (2018) · Scientific Reports · doi:10.1038/s41598-017-14908-6 · Cited by 42
LRP130, a single-stranded DNA/RNA-binding protein, localizes at the outer nuclear and endoplasmic reticulum membrane, and interacts with mRNA in vivo
Tsuchiya, N., Fukuda, H., Nakashima, K., Nagao, M., Sügimura, T., & Nakagama, H. (2004) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2004.03.103 · Cited by 42
Fragile X premutation in women: recognizing the health challenges beyond primary ovarian insufficiency
Hoyos, L. R., & Thakur, M. (2016) · Journal of Assisted Reproduction and Genetics · doi:10.1007/s10815-016-0854-6 · Cited by 41
Mechanisms of Long Non-Coding RNAs in the Assembly and Plasticity of Neural Circuitry
Wang, A., Wang, J., Liu, Y., & Zhou, Y. (2017) · Frontiers in Neural Circuits · doi:10.3389/fncir.2017.00076 · Cited by 42
The RNA binding protein FMRP: new connections and missing links
Schaeffer, C., Beaulande, M., Ehresmann, C., Ehresmann, B., & Moine, H. (2003) · Biology of the Cell · doi:10.1016/s0248-4900(03)00037-6 · Cited by 44
Serotonin dysregulation in Fragile X Syndrome: implications for treatment
Hanson, A. C., & Hagerman, R. J. (2014) · Intractable & Rare Diseases Research · doi:10.5582/irdr.2014.01027 · Cited by 40
Post-translational modifications of the Fragile X Mental Retardation Protein in neuronal function and dysfunction
Prieto, M., Folci, A., & Martin, S. S. (2019) · Molecular Psychiatry · doi:10.1038/s41380-019-0629-4 · Cited by 44
Occupancy and synergistic activation of the FMR1 promoter by Nrf-1 and Sp1 in vivo
Smith, K. T. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh172 · Cited by 40
Mouse‐centric comparative transcriptomics of protein coding and non‐coding RNAs
Suzuki, M., & Hayashizaki, Y. (2004) · BioEssays · doi:10.1002/bies.20084 · Cited by 40
A normal genetic variation modulates synaptic MMP-9 protein levels and the severity of schizophrenia symptoms.
Lepeta, K., Purzycka, K. J., Pachulska‐Wieczorek, K., Mitjans, M., Begemann, M., Vafadari, B., Bijata, K., Adamiak, R. W., Ehrenreich, H., Dziembowska, M., et al. (2017) · EMBO Molecular Medicine · doi:10.15252/emmm.201707723 · Cited by 39
Fear extinction is regulated by the activity of long noncoding RNAs at the synapse
Liau, W., Zhao, Q., Bademosi, A. T., Gormal, R. S., Gong, H., Marshall, P. R., Periyakaruppiah, A., Madugalle, S. U., Zajaczkowski, E. L., Leighton, L. J., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-43535-1 · Cited by 38
The Presynaptic Microtubule Cytoskeleton in Physiological and Pathological Conditions: Lessons from Drosophila Fragile X Syndrome and Hereditary Spastic Paraplegias
Bodaleo, F. J., & González-Billault, C. E. (2016) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2016.00060 · Cited by 40
Interneuron Dysfunction in Syndromic Autism: Recent Advances
Takano, T. (2015) · Developmental Neuroscience · doi:10.1159/000434638 · Cited by 41
The Fragile X Mental Retardation Protein in Circadian Rhythmicity and Memory Consolidation
Gatto, C. L., & Broadie, K. S. (2009) · Molecular Neurobiology · doi:10.1007/s12035-009-8057-0 · Cited by 38
Elevated levels of FMRP-target MAP1B impair human and mouse neuronal development and mouse social behaviors via autophagy pathway
Guo, Y., Shen, M., Dong, Q., Méndez-Albelo, N. M., Huang, S. X., Sirois, C. L., Le, J., Li, M., Jarzembowski, E. D., Schoeller, K. A., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-39337-0 · Cited by 42
Lithium: A Promising Treatment for Fragile X Syndrome
Liu, Z., & Smith, C. B. (2014) · ACS Chemical Neuroscience · doi:10.1021/cn500077p · Cited by 39
Above genetics: Lessons from cerebral development in autism
Williams, E., & Casanova, M. F. (2011) · Translational Neuroscience · doi:10.2478/s13380-011-0016-3 · Cited by 38
Perturbation of dendritic protrusions in intellectual disability
Levenga, J., & Willemsen, R. (2012) · Progress in brain research · doi:10.1016/b978-0-444-54299-1.00008-x · Cited by 38
Identification of the RGG Box Motif in Shadoo: RNA-Binding and Signaling Roles?
Corley, S., & Gready, J. E. (2008) · Bioinformatics and Biology Insights · doi:10.4137/bbi.s1075 · Cited by 37
Post-transcriptional control of neurofilaments in development and disease
Thyagarajan, A., Strong, M. J., & Szaro, B. G. (2007) · Experimental Cell Research · doi:10.1016/j.yexcr.2007.02.014 · Cited by 37
Multiple Roles of Alu-Related Noncoding RNAs
Berger, A., & Strub, K. (2010) · Progress in molecular and subcellular biology · doi:10.1007/978-3-642-16502-3_6 · Cited by 35
Non-coding mechanisms of local mRNA translation in neuronal dendrites
Weiß, K., Antoniou, A., & Schratt, G. M. (2015) · European Journal of Cell Biology · doi:10.1016/j.ejcb.2015.05.011 · Cited by 35
Characterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS
Biscarini, S., Capauto, D., Peruzzi, G., Lü, L., Colantoni, A., Santini, T., Shneider, N. A., Caffarelli, E., Laneve, P., & Bozzoni, I. (2018) · Stem Cell Research · doi:10.1016/j.scr.2018.01.037 · Cited by 35
Fragile X Syndrome and Alzheimers Disease: Another Story About APP and β -Amyloid
Malter, J. S., Ray, B. C., Westmark, P. R., & Westmark, C. J. (2010) · Current Alzheimer Research · doi:10.2174/156720510791050957 · Cited by 34
Elevated activation of CaMKIIα in the CPEB3-knockout hippocampus impairs a specific form of NMDAR-dependent synaptic depotentiation
Huang, W.-H., Chao, H., Tsai, L.-Y., Chung, M.-H., & Huang, Y. (2014) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2014.00367 · Cited by 31
A microRNA cluster in the Fragile‐X region expressed during spermatogenesis targets FMR1
Ramaiah, M., Tan, K., Plank, T. M., Song, H., Chousal, J. N., Jones, S. H., Shum, E. Y., Sheridan, S. D., Peterson, K. J., Gromoll, J., et al. (2018) · EMBO Reports · doi:10.15252/embr.201846566 · Cited by 33
Fragile X syndrome and model organisms: identifying potential routes of therapeutic intervention
Bhogal, B., & Jongens, T. A. (2010) · Disease Models & Mechanisms · doi:10.1242/dmm.002006 · Cited by 36
From Learning to Memory: What Flies Can Tell Us about Intellectual Disability Treatment
Androschuk, A., Al-Jabri, B. A., & Bolduc, F. V. (2015) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2015.00085 · Cited by 33
Long non-coding RNAs in Alzheimer's disease
Zhang, Z. (2015) · Current Topics in Medicinal Chemistry · doi:10.2174/1568026615666150813142956 · Cited by 33
FMRP modulates the Wnt signalling pathway in glioblastoma
Pedini, G., Buccarelli, M., Bianchi, F., Pacini, L., Cencelli, G., D’Alessandris, Q. G., Martini, M., Giannetti, S., Sasso, F., Melocchi, V., et al. (2022) · Cell Death and Disease · doi:10.1038/s41419-022-05019-w · Cited by 33
Self-citation
Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome
Qiu, L.-F., Lu, T., Hu, X.-L., Yi, Y., Liao, W., & Xiong, Z. (2008) · Cerebral Cortex · doi:10.1093/cercor/bhn163 · Cited by 33
The Conserved, Disease-Associated RNA Binding Protein dNab2 Interacts with the Fragile X Protein Ortholog in Drosophila Neurons
Bienkowski, R. S., Banerjee, A., Rounds, J. C., Rha, J., Omotade, O. F., Groß, C., Morris, K. J., Leung, S. W., Pak, C., Jones, S. K., et al. (2017) · Cell Reports · doi:10.1016/j.celrep.2017.07.038 · Cited by 32
MD and Docking Studies Reveal That the Functional Switch of CYFIP1 is Mediated by a Butterfly-like Motion
Marino, D. D., Chillemi, G., Rubeis, S. D., Tramontano, A. L., Achsel, T., & Bagni, C. (2015) · Journal of Chemical Theory and Computation · doi:10.1021/ct500431h · Cited by 33
Self-citation
A kinesin adapter directly mediates dendritic mRNA localization during neural development in mice
Wu, H., Zhou, J., Zhu, T., Cohen, I., & Dictenberg, J. B. (2020) · Journal of Biological Chemistry · doi:10.1074/jbc.ra118.005616 · Cited by 33
On the functional relevance of spatiotemporally-specific patterns of experience-dependent long noncoding RNA expression in the brain
Liau, W., Samaddar, S., Banerjee, S., & Bredy, T. W. (2021) · RNA Biology · doi:10.1080/15476286.2020.1868165 · Cited by 27
Fragile X mental retardation protein is required for programmed cell death and clearance of developmentally-transient peptidergic neurons
Gatto, C. L., & Broadie, K. S. (2011) · Developmental Biology · doi:10.1016/j.ydbio.2011.05.001 · Cited by 32
Molecular Insights into Mental Retardation: Multiple Functions for the Fragile X Mental Retardation Protein?
Zalfa, F., & Bagni, C. (2004) · Current Issues in Molecular Biology · doi:10.21775/cimb.006.073 · Cited by 33
Self-citation
Excessive proteostasis contributes to pathology in fragile X syndrome
Louros, S. R., Seo, S. S., Maio, B., Martinez‐Gonzalez, C., Gonzalez‐Lozano, M. A., Muscas, M., Verity, N. C., Wills, J. C., Li, K. W., Nolan, M. F., et al. (2022) · Neuron · doi:10.1016/j.neuron.2022.11.012 · Cited by 32
Arc ubiquitination in synaptic plasticity
Mabb, A. M., & Ehlers, M. (2017) · Seminars in Cell and Developmental Biology · doi:10.1016/j.semcdb.2017.09.009 · Cited by 29
Fragile X and APP: a Decade in Review, a Vision for the Future
Westmark, C. J. (2018) · Molecular Neurobiology · doi:10.1007/s12035-018-1344-x · Cited by 31
FMRP regulates presynaptic localization of neuronal voltage gated calcium channels
Ferron, L., Novazzi, C. G., Pilch, K. S., Moreno, C., Ramgoolam, K., & DOLPHIN, A. C. (2020) · Neurobiology of Disease · doi:10.1016/j.nbd.2020.104779 · Cited by 31
Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome
Tessier, C. R., & Broadie, K. S. (2011) · Results and problems in cell differentiation · doi:10.1007/978-3-642-21649-7_7 · Cited by 31
Dysregulated mTORC1-Dependent Translational Control: From Brain Disorders to Psychoactive Drugs
Santini, E., & Klann, E. (2011) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2011.00076 · Cited by 34
Chronic minocycline treatment improves hippocampal neuronal structure, NMDA receptor function, and memory processing in Fmr1 knockout mice
Yau, S., Bettio, L. E. B., Vetrici, M. A., Truesdell, A., Chiu, C., Chiu, J., Truesdell, E., & Christie, B. R. (2018) · Neurobiology of Disease · doi:10.1016/j.nbd.2018.01.014 · Cited by 30
Drug repositioning screening identifies etravirine as a potential therapeutic for friedreich's ataxia
Alfedi, G., Luffarelli, R., Condò, I., Pedini, G., Mannucci, L., Massaro, D. S., Benini, M., Toschi, N., Alaimo, G., Panarello, L., et al. (2019) · Movement Disorders · doi:10.1002/mds.27604 · Cited by 31
Disruption of striatal-enriched protein tyrosine phosphatase (STEP) function in neuropsychiatric disorders
Karasawa, T., & Lombroso, P. J. (2014) · Neuroscience Research · doi:10.1016/j.neures.2014.08.018 · Cited by 30
Arc 3′ UTR Splicing Leads to Dual and Antagonistic Effects in Fine-Tuning Arc Expression Upon BDNF Signaling
Paolantoni, C., Ricciardi, S., Paolis, V. D., Okenwa, C., Catalanotto, C., Ciotti, M. T., Cattaneo, A., Cogoni, C., & Giorgi, C. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00145 · Cited by 26
Fragile X Mental Retardation Protein positively regulates PKA anchor Rugose and PKA activity to control actin assembly in learning/memory circuitry
Sears, J. C., Choi, W. J., & Broadie, K. S. (2019) · Neurobiology of Disease · doi:10.1016/j.nbd.2019.02.004 · Cited by 30
The HNF4α-BC200-FMR1–Positive Feedback Loop Promotes Growth and Metastasis in Invasive Mucinous Lung Adenocarcinoma
Chen, X., Zhao, Y., Wang, D., Lin, Y., Hou, J., Xu, X., Wu, J., Zhong, L., Zhou, Y., Shen, J., et al. (2021) · Cancer Research · doi:10.1158/0008-5472.can-21-0980 · Cited by 30
Corticosterone-induced enhancement of memory and synaptic Arc protein in the medial prefrontal cortex
McReynolds, J. R., Holloway-Erickson, C. M., Parmar, T. U., & McIntyre, C. K. (2014) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2014.02.007 · Cited by 29
Maintenance of CCL5 mRNA stores by post‐effector and memory CD8 T cells is dependent on transcription and is coupled to increased mRNA stability
Marçais, A., Tomkowiak, M., Walzer, T., Coupet, C., Ravel‐Chapuis, A., & Marvel, J. (2006) · European Journal of Immunology · doi:10.1002/eji.200636259 · Cited by 29
The integrated stress response in brain diseases: A double-edged sword for proteostasis and synapses
Lockshin, E. R., & Calakos, N. (2024) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2024.102886 · Cited by 31
Environmental Enrichment Reveals Effects of Genotype on Hippocampal Spine Morphologies in the Mouse Model of Fragile X Syndrome
Lauterborn, J. C., Jafari, M., Babayan, A. H., & Gall, C. M. (2013) · Cerebral Cortex · doi:10.1093/cercor/bht249 · Cited by 31

Authors: Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., & Bagni, C.
Affiliations: University of Rome Tor Vergata (IT)
Erasmus MC (NL)
Corresponding author: Bagni, Claudia
ORCID: Zalfa, Francesca
Reis, Surya
Bagni, Claudia
Venue: Cell
Publisher: Elsevier BV
Published: 2003-02
Volume: 112
Issue: 3
Pages: 317-327
ISSN: 0092-8674 (ISSN-L)
DOI: 10.1016/s0092-8674(03)00079-5
OpenAlex: W2002364516
Cited by: 676
References: 65
Citations by year: 2026: 4 · 2025: 13 · 2024: 14 · 2023: 13 · 2022: 9 · 2021: 21 · 2020: 19 · 2019: 27 · 2018: 28 · 2017: 36 · 2016: 19 · 2015: 33 · 2014: 39 · 2013: 31 · 2012: 36
FWCI: 26.25
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 3819 d)
Locations: Cell (publishedVersion)
PubMed (publishedVersion)
EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
Lirias (submittedVersion, other-oa)
EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
Cineca Institutional Research Information System (Tor Vergata University) (submittedVersion)
Funders: Eunice Kennedy Shriver National Institute of Child Health and Human Development (5R01 HD 38038, R01HD038038)
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, BC1 RNA, protein synthesis inhibition, synaptic dysfunction, intellectual disability, RNA-binding proteins
MeSH terms: Animals, Base Sequence, Brain, Dendrites, Fragile X Syndrome, Humans, Molecular Sequence Data, Nerve Tissue Proteins, Synaptic Transmission, Protein Binding, Ribonucleoproteins, RNA, Messenger, Synapses, Protein Biosynthesis, Tumor Cells, Cultured, Molecular Structure, Genes, Suppressor, RNA-Binding Proteins, Mice, Knockout, RNA, Small Cytoplasmic, Macromolecular Substances, Mice, Fragile X Messenger Ribonucleoprotein 1

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

The Fragile X Syndrome Protein FMRP Associates with BC1 RNA and Regulates the Translation of Specific mRNAs at Synapses

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Self-check

Citations

Cites

Cited by

Related

Metadata

Record

About these checks