Lovastatin, Not Simvastatin, Corrects Core Phenotypes in the Fragile X Mouse Model
Open accessPublished version of a preprint
Abstract
The cholesterol-lowering drug lovastatin corrects neurological phenotypes in animal models of fragile X syndrome (FX), a commonly identified genetic cause of autism and intellectual disability (ID). The therapeutic efficacy of lovastatin is being tested in clinical trials for FX; however, the structurally similar drug simvastatin has been proposed as an alternative due to an increased potency and brain penetrance. Here, we perform a side-by-side comparison of the effects of lovastatin and simvastatin treatment on two core phenotypes in Fmr1 -/y mice versus WT littermates: excessive hippocampal protein synthesis and susceptibility to audiogenic seizures (AGSs). We find that simvastatin does not correct excessive hippocampal protein synthesis in the Fmr1 -/y hippocampus at any dose tested. In fact, simvastatin significantly increases protein synthesis in both Fmr1 -/y and WT. Moreover, injection of simvastatin does not reduce AGS in the Fmr1 -/y mouse, while lovastatin significantly reduces AGS incidence and severity versus vehicle-treated animals. These results show that unlike lovastatin, simvastatin does not correct core phenotypes in the Fmr1 -/y mouse model.
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
Cites
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?
Pharmacological inhibition of the MVA pathway corrects excessive hippocampal protein synthesis and susceptibility to audiogenic seizures in FXS mice.
“it has been demonstrated that the pharmacological inhibition of the MVA pathway corrects the excessive hippocampal protein synthesis and susceptibility to audiogenic seizures displayed by FXS mice, suggesting a potential role of the cholesterol biosynthetic pathway in this pathology [25].”
From Brain Cholesterol Biosynthetic Pathway Is Altered in a Preclinical Model of Fragile X Syndrome (2022) · cites this paper as [25]
The Results (no page available) show that lovastatin normalizes excessive hippocampal protein synthesis and reduces audiogenic seizure incidence in Fmr1-/y mice. But the same sections report that simvastatin does not correct either phenotype, so the source does not support the unqualified claim that pharmacological inhibition of the mevalonate pathway does so. The Discussion proposes an effect through ERK1/2 signaling rather than establishing inhibition of cholesterol biosynthesis as the corrective mechanism. (LLM, conf=0.88)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
A Simple Practice Guide for Dose Conversion between Animals and Human
Nair, A., & Jacob, S. (2016) · Journal of Basic and Clinical Pharmacy · doi:10.4103/0976-0105.177703 · Cited by 6,174 · see citing works
FMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism
Darnell, J. C., Van Driesche, S. J., Zhang, C., Hung, K. Y. S., Mele, A., Fraser, C. E., Stone, E. F., Chen, C., Fak, J. J., Chi, S. W., et al. (2011) · Cell · doi:10.1016/j.cell.2011.06.013 · Cited by 2,072 · see citing works
PLEIOTROPIC EFFECTS OF STATINS
Liao, J. K., & Laufs, U. (2005) · Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev.pharmtox.45.120403.095748 · Cited by 1,994 · see citing works
The mGluR theory of fragile X mental retardation
Bear, M. F., Huber, K. M., & Warren, S. T. (2004) · Trends in Neurosciences · doi:10.1016/j.tins.2004.04.009 · Cited by 1,580 · see citing works
Chemical, pharmacokinetic and pharmacodynamic properties of statins: an update
Schächter, M. (2004) · Fundamental and Clinical Pharmacology · doi:10.1111/j.1472-8206.2004.00299.x · Cited by 1,216 · see citing works
Jones, P., Kafonek, S., Laurora, I., & Hunninghake, D. (1998) · The American Journal of Cardiology · doi:10.1016/s0002-9149(97)00965-x · Cited by 1,067 · see citing works
Correction of Fragile X Syndrome in Mice
Dölen, G., Osterweil, E., Rao, B. S. S., Smith, G. B., Auerbach, B. D., Chattarji, S., & Bear, M. F. (2007) · Neuron · doi:10.1016/j.neuron.2007.12.001 · Cited by 979 · see citing works
Self-citationFMR1 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 741 · see citing works
Advances in the Treatment of Fragile X Syndrome
Hagerman, R. J., Berry-Kravis, E., Kaufmann, W. E., Ono, M. Y., Tartaglia, N., Lachiewicz, A., Kronk, R., Delahunty, C., Hessl, D., Visootsak, J., et al. (2009) · Pediatrics · doi:10.1542/peds.2008-0317 · Cited by 596 · see citing works
Dysregulation of mTOR signaling in fragile X syndrome
Sharma, A., Hoeffer, C. A., Takayasu, Y., Miyawaki, T., McBride, S. M., Klann, E., & Zukin, R. S. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.3696-09.2010 · Cited by 557 · see citing works
Genetic and Pharmacological Suppression of Oncogenic Mutations in RAS Genes of Yeast and Humans
Schafer, W. R., Kim, R., Sterne, R. E., Thorner, J. W., Kim, S., & Rine, J. (1989) · Science · doi:10.1126/science.2569235 · Cited by 530 · see citing works
Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP
Yan, Q. J., Rammal, M., Tranfaglia, M., & Bauchwitz, R. P. (2005) · Neuropharmacology · doi:10.1016/j.neuropharm.2005.06.004 · Cited by 521 · see citing works
Signalling to translation: How signal transduction pathways control the protein synthetic machinery
Proud, C. G. (2007) · Biochemical Journal · doi:10.1042/bj20070024 · Cited by 520 · see citing works
Chronic Pharmacological mGlu5 Inhibition Corrects Fragile X in Adult Mice
Michalon, A., Sidorov, M., Ballard, T. M., Ozmen, L., Spooren, W., Wettstein, J. G., Jaeschke, G., Bear, M. F., & Lindemann, L. (2012) · Neuron · doi:10.1016/j.neuron.2012.03.009 · Cited by 489 · see citing works
Muddashetty, R. S., Kelić, S., Gross, C., Xu, M., & Bassell, G. J. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.0937-07.2007 · Cited by 421 · see citing works
Li, W., Cui, Y., Kushner, S. A., Brown, R. A. M., Jentsch, J. D., Frankland, P. W., Cannon, T. D., & Silva, A. J. (2005) · Current Biology · doi:10.1016/j.cub.2005.09.043 · Cited by 403 · see citing works
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 · see citing works
Self-citationDrug development for neurodevelopmental disorders: lessons learned from fragile X syndrome
Berry-Kravis, E. M., Lindemann, L., Jønch, A. E., Apostol, G., Bear, M. F., Carpenter, R. L., Crawley, J. N., Curie, A., Des Portes, V., Hossain, F., et al. (2018) · Nature Reviews Drug Discovery · doi:10.1038/nrd.2017.221 · Cited by 336 · see citing works
Audiogenic Seizures Susceptibility in Transgenic Mice with Fragile X Syndrome
Musumeci, S. A., Bosco, P., Calabrese, G., Bakker, C., De Sarro, G. B., Elia, M., Ferri, R., & Oostra, B. A. (2000) · Epilepsia · doi:10.1111/j.1528-1157.2000.tb01499.x · Cited by 289 · see citing works
Epilepsy in fragile X syndrome
Berry‐Kravis, E. (2002) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.2002.tb00277.x · Cited by 271 · see citing works
Qin, M., Kang, J., Burlin, T. V., Jiang, C., & Smith, C. B. (2005) · The Journal of Neuroscience · doi:10.1523/jneurosci.0093-05.2005 · Cited by 263 · see citing works
Targeting the endocannabinoid system in the treatment of fragile X syndrome
Busquets-Garcia, A., Gomis-González, M., Guegan, T., Agustín-Pavón, C., Pastor, A., Mato, S., Pérez-Samartín, A., Matute, C., de la Torre, R., Dierssen, M., et al. (2013) · Nature Medicine · doi:10.1038/nm.3127 · Cited by 241 · see citing works
Osterweil, E. K., Chuang, S.-C., Chubykin, A. A., Sidorov, M., Bianchi, R., Wong, R. K. S., & Bear, M. F. (2013) · Neuron · doi:10.1016/j.neuron.2012.01.034 · Cited by 230 · see citing works
Self-citationNeuvonen, P. J., Backman, J. T., & Niemi, M. (2008) · Clinical Pharmacokinetics · doi:10.2165/00003088-200847070-00003 · Cited by 217 · see citing works
A phenotypic and molecular characterization of the <i>fmr1‐tm1Cgr</i> Fragile X mouse
Yan, Q. J., Asafo‐Adjei, P. K., Arnold, H. M., Brown, R. E., & Bauchwitz, R. P. (2004) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2004.00087.x · Cited by 213 · see citing works
Chronic Administration of Statins Alters Multiple Gene Expression Patterns in Mouse Cerebral Cortex
Johnson‐Anuna, L. N., Eckert, G. P., Keller, J. H., Igbavboa, U., Franke, C., Fechner, T., Schubert‐Zsilavecz, M., Karas, M., Müller, W. E., & Wood, W. G. (2005) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.104.075028 · Cited by 199 · see citing works
Statin inhibition of HMG-CoA reductase: a 3-dimensional view
Istvan, E. S. (2003) · Atherosclerosis Supplements · doi:10.1016/s1567-5688(03)00003-5 · Cited by 196 · see citing works
Effect of Simvastatin on Cognitive Functioning in Children With Neurofibromatosis Type 1
Krab, L. C. (2008) · JAMA · doi:10.1001/jama.300.3.287 · Cited by 194 · see citing works
Lozano, R., Rosero, C. A., & Hagerman, R. J. (2014) · Intractable & Rare Diseases Research · doi:10.5582/irdr.2014.01022 · Cited by 186 · see citing works
The Ras-related Protein Rheb Is Farnesylated and Antagonizes Ras Signaling and Transformation
Clark, G. J., Kinch, M. S., Rogers-Graham, K., Sebti, S. M., Hamilton, A. D., & Der, C. J. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.16.10608 · Cited by 177 · see citing works
Basso, A. D., Mirza, A., Liu, G., Long, B. J., Bishop, W. R., & Kirschmeier, P. T. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m503763200 · Cited by 162 · see citing works
Schaefer, E. J., McNamara, J. R., Tayler, T., Daly, J. A., Gleason, J. L., SEMAN, L. J., Ferrari, A., & Rubenstein, J. J. (2003) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2003.09.008 · Cited by 157 · see citing works
Simvastatin enhances learning and memory independent of amyloid load in mice
Li, L., Cao, D., Kim, H., Lester, R. A. J., & Fukuchi, K. (2006) · Annals of Neurology · doi:10.1002/ana.21053 · Cited by 150 · see citing works
van der Vaart, T., Plasschaert, E., Rietman, A. B., Renard, M., Oostenbrink, R., Vogels, A., de Wit, M. Y., Descheemaeker, M., Vergouwe, Y., Catsman‐Berrevoets, C. E., et al. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70227-8 · Cited by 128 · see citing works
Tsuji, A., Saheki, A., Tamai, I., & Terasaki, T. (1993) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1016/s0022-3565(25)39496-6 · Cited by 106 · see citing works
Randomized placebo-controlled study of lovastatin in children with neurofibromatosis type 1
Payne, J. M., Barton, B., Ullrich, N. J., Cantor, A. B., Hearps, S. J. C., Cutter, G., Rosser, T. L., Walsh, K. S., Gioia, G. A., Wolters, P. L., et al. (2016) · Neurology · doi:10.1212/wnl.0000000000003435 · Cited by 102 · see citing works
Cell-Type-Specific Translation Profiling Reveals a Novel Strategy for Treating Fragile X Syndrome
Thomson, S. R., Seo, S. S., Barnes, S. A., Louros, S. R., Muscas, M., Dando, O., Kirby, C., Wyllie, D. J. A., Hardingham, G. E., Kind, P. C., et al. (2017) · Neuron · doi:10.1016/j.neuron.2017.07.013 · Cited by 100 · see citing works
Self-citationHiggins, J. W., Bao, J., Ke, A. B., Manro, J. R., Fallon, J. K., Smith, P. C., & Zamek‐Gliszczynski, M. J. (2013) · Drug Metabolism and Disposition · doi:10.1124/dmd.113.054783 · Cited by 96 · see citing works
Sebti, S. M., Tkalcevic, G. T., & Jani, J. P. (1991) · Cancer Communications · doi:10.3727/095535491820873371 · Cited by 92 · see citing works
Stoppel, L. J., Kazdoba, T. M., Schaffler, M. D., Preza, A. R., Heynen, A., Crawley, J. N., & Bear, M. F. (2018) · Neuropsychopharmacology · doi:10.1038/npp.2017.236 · Cited by 92 · see citing works
Wang, X., Snape, M., Klann, E., Stone, J. G., Singh, A., Petersen, R. B., Castellani, R. J., Casadesús, G., Smith, M. A., & Zhu, X. (2012) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2012.07722.x · Cited by 91 · see citing works
Simvastatin protects neurons from cytotoxicity by up‐regulating Bcl‐2 mRNA and protein
Johnson‐Anuna, L. N., Eckert, G. P., Franke, C., Igbavboa, U., Müller, W. E., & Wood, W. G. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2006.04375.x · Cited by 79 · see citing works
Chuncheng, X., Sun, J., Qiao, W., Lu, D., Wei, L., Meng, N., Song, Y., Hou, X., & Lin, Z. (2011) · PLoS ONE · doi:10.1371/journal.pone.0024966 · Cited by 78 · see citing works
Asiminas, A., Jackson, A. D., Louros, S. R., Till, S. M., Spano, T., Dando, O., Bear, M. F., Chattarji, S., Hardingham, G. E., Osterweil, E. K., et al. (2019) · Science Translational Medicine · doi:10.1126/scitranslmed.aao0498 · Cited by 78 · see citing works
Self-citationEffect of lovastatin on behavior in children and adults with fragile X syndrome: An open‐label study
Çaku, A., Pellerin, D., Bouvier, P., Riou, E., & Corbin, F. (2014) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36750 · Cited by 77 · see citing works
Ramírez, C. D., Tercero, I., Pineda, A., & Burgos, J. S. (2011) · Journal of Alzheimer s Disease · doi:10.3233/jad-2010-101653 · Cited by 70 · see citing works
Simvastatin enhances hippocampal long-term potentiation in C57BL/6 mice
Mans, R. A., Chowdhury, N., Cao, D., McMahon, L. L., & Li, L. (2009) · Neuroscience · doi:10.1016/j.neuroscience.2009.12.062 · Cited by 67 · see citing works
Gonzalez, D., Tomasek, M. P., Hays, S. A., Sridhar, V., Ammanuel, S. G., Chang, C.-W., Pawlowski, K. S., Huber, K. M., & Gibson, J. R. (2019) · Journal of Neuroscience · doi:10.1523/jneurosci.0886-19.2019 · Cited by 64 · see citing works
Lim, J. H., Lee, J., Lee, Y. H., Choi, I. Y., Oh, Y., Kim, H., Park, J., & Kim, W. (2006) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2006.03715.x · Cited by 60 · see citing works
Lithium treatment alleviates impaired cognition in a mouse model of fragile X syndrome
King, M. K., & Jope, R. S. (2013) · Genes Brain & Behavior · doi:10.1111/gbb.12071 · Cited by 57 · see citing works
Concepts in Ras-directed therapy
Kloog, Y., Cox, A. D., & Sinensky, M. S. (1999) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.8.12.2121 · Cited by 53 · see citing works
A randomized placebo‐controlled lovastatin trial for neurobehavioral function in neurofibromatosis I
Bearden, C. E., Hellemann, G., Rosser, T. L., Montojo, C. A., Jonas, R. K., Enrique, N., Pacheco, L., Hussain, S. A., Wu, J. Y., Ho, J. S., et al. (2016) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.288 · Cited by 53 · see citing works
Pellerin, D., Çaku, A., Fradet, M., Bouvier, P., Dubé, J., & Corbin, F. (2016) · Biomarkers · doi:10.3109/1354750x.2016.1160289 · Cited by 45 · see citing works
Efficacy and Tolerability of Lovastatin in 3390 Women with Moderate Hypercholesterolemia
Bradford, R. H., Downton, M., Chremos, A. N., Langendorfer, A., Stinnett, S. S., Nash, D. T., Mantell, G., & Shear, C. L. (1993) · Annals of Internal Medicine · doi:10.7326/0003-4819-118-11-199306010-00004 · Cited by 39 · see citing works
Statins and the brain: New perspective for old drugs
Ling, Q., & Tejada-Simon, M. V. (2015) · Progress in Neuro-Psychopharmacology and Biological Psychiatry · doi:10.1016/j.pnpbp.2015.11.013 · Cited by 38 · see citing works
Lovastatin blocks N-ras oncogene-induced neuronal differentiation.
Mendola, C. E., & Backer, J. (1990) · PubMed · Cited by 35 · see citing works
Decreased exposure of simvastatin and simvastatin acid in a rat model of type 2 diabetes
Xu, D., Li, F., Zhang, M., Zhang, J., Liu, C., Hu, M., Zhong, Z., Jia, L.-L., Wang, D., Wu, J., et al. (2014) · Acta Pharmacologica Sinica · doi:10.1038/aps.2014.39 · Cited by 33 · see citing works
van de Steeg, E., Kleemann, R., Jansen, H. T., van Duyvenvoorde, W., Offerman, E. H., Wortelboer, H. M., & DeGroot, J. (2013) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.113.208595 · Cited by 30 · see citing works
The analytical determination of isoprenoid intermediates from the mevalonate pathway
Nürenberg, G., & Volmer, D. A. (2011) · Analytical and Bioanalytical Chemistry · doi:10.1007/s00216-011-5262-2 · Cited by 24 · see citing works
Fragile X syndrome: From genetics to targeted treatment
Willemsen, R., & Kooy, R. F. (2017) · Cited by 22 · see citing works
Considerations for Clinical Therapeutic Development of Statins for Neurodevelopmental Disorders
Ottenhoff, M. J., Krab, L. C., & Elgersma, Y. (2020) · eNeuro · doi:10.1523/eneuro.0392-19.2020 · Cited by 9 · see citing works
Flagged first, then by citations
Research works printed but not linked: 1
- Jones P, Kafonek S, Laurora I, Hunninghake D (1998) Comparative dose efficacy study of atorvastatin versus simvastatin, pravastatin, lovastatin, and fluvastatin in patients with hypercholesterolemia (the CURVES study). Am J Cardiol 81: 582– 587. 9514454
Cited by
Sorted by citations
ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome
Licznerski, P., Park, H., Rolyan, H., Chen, R., Mnatsakanyan, N., Miranda, P., Graham, M. M., Wu, J., Cruz-Reyes, N., Mehta, N. A., et al. (2020) · Cell · doi:10.1016/j.cell.2020.07.008 · Cited by 114 · see citing works
Gonzalez, D., Tomasek, M. P., Hays, S. A., Sridhar, V., Ammanuel, S. G., Chang, C.-W., Pawlowski, K. S., Huber, K. M., & Gibson, J. R. (2019) · Journal of Neuroscience · doi:10.1523/jneurosci.0886-19.2019 · Cited by 64 · see citing works
Mechanisms underlying auditory processing deficits in Fragile X syndrome
McCullagh, E., Rotschafer, S., Auerbach, B. D., Klug, A., Kaczmarek, L. K., Cramer, K. S., Kulesza, R., Razak, K. A., Lovelace, J., Lu, Y., et al. (2020) · The FASEB Journal · doi:10.1096/fj.201902435r · Cited by 64 · see citing works
Emerging Gene and Small Molecule Therapies for the Neurodevelopmental Disorder Angelman Syndrome
Copping, N. A., McTighe, S. M., Fink, K. D., & Silverman, J. L. (2021) · Neurotherapeutics · doi:10.1007/s13311-021-01082-x · Cited by 39 · see citing works
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 30 · see citing works
Self-citationReyes, S. T., Deacon, R. M. J., Guo, S. G., Altimiras, F. J., Castillo, J. B., van der Wildt, B., Morales, A. P., Park, J. H., Klamer, D., Rosenberg, J., et al. (2021) · Scientific Reports · doi:10.1038/s41598-021-94079-7 · Cited by 22 · see citing works
Dysregulated translational control in brain disorders: from genes to behavior
Wiebe, S. A., Nagpal, A., & Sonenberg, N. (2020) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2020.05.005 · Cited by 24 · see citing works
Champigny, C., Morin-Parent, F., Bellehumeur-Lefebvre, L., Çaku, A., Lepage, J.-F., & Corbin, F. (2022) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.762967 · Cited by 16 · see citing works
A Highly Selective MNK Inhibitor Rescues Deficits Associated with Fragile X Syndrome in Mice
Shukla, T. N., de la Peña, J. B., Perish, J. M., Ploski, J. E., Stumpf, C. R., Webster, K. R., Thorn, C. A., & Campbell, Z. T. (2020) · Neurotherapeutics · doi:10.1007/s13311-020-00932-4 · Cited by 16 · see citing works
Lacroix, A., Proteau-Lemieux, M., Cote, S. R., Near, J., Hui, S. C. N., Edden, R. A. E., Lippé, S., Çaku, A., Corbin, F., & Lepage, J. (2022) · Neurobiology of Disease · doi:10.1016/j.nbd.2022.105881 · Cited by 14 · see citing works
FMRP activity and control of Csw/SHP2 translation regulate MAPK-dependent synaptic transmission
Leahy, S. N., Song, C., Vita, D. J., & Broadie, K. S. (2023) · PLoS Biology · doi:10.1371/journal.pbio.3001969 · Cited by 14 · see citing works
Fragile X Syndrome: Lessons Learned and What New Treatment Avenues Are on the Horizon
Hagerman, R. J., & Hagerman, P. J. (2021) · The Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev-pharmtox-052120-090147 · Cited by 12 · see citing works
Brain Cholesterol Biosynthetic Pathway Is Altered in a Preclinical Model of Fragile X Syndrome
Parente, M., Tonini, C., Buzzelli, V., Carbone, E., Trezza, V., & Pallottini, V. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23063408 · Cited by 11 · see citing works
“cal inhibition of the MVA pathway corrects the excessive hippocampal protein synthesis and susceptibility to audiogenic seizures displayed by FXS mice, suggesting a potential role of the cholesterol biosynthetic pathway in this pathology [25].”
BackgroundFragile X Syndrome and Targeted Treatments
Tassanakijpanich, N., Cabal-Herrera, A. M., Salcedo‐Arellano, M. J., & Hagerman, R. J. (2020) · Journal of Biomedicine and Translational Research · doi:10.14710/jbtr.v6i1.7321 · Cited by 10 · see citing works
Considerations for Clinical Therapeutic Development of Statins for Neurodevelopmental Disorders
Ottenhoff, M. J., Krab, L. C., & Elgersma, Y. (2020) · eNeuro · doi:10.1523/eneuro.0392-19.2020 · Cited by 9 · see citing works
An iPSC-derived astrocyte model of fragile X syndrome exhibits dysregulated cholesterol homeostasis
Talvio, K., Wagner, V. A., Minkeviciene, R., Kirkwood, J. S., Kulinich, A. O., Umemori, J., Bhatia, A., Hur, M., Käkelä, R., Ethell, I. M., et al. (2023) · Communications Biology · doi:10.1038/s42003-023-05147-9 · Cited by 8 · see citing works
Astrocytes in fragile X syndrome
Talvio, K., & Castrén, M. L. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1322541 · Cited by 8 · see citing works
A Differential Effect of Lovastatin versus Simvastatin in Neurodevelopmental Disorders
Muscas, M., Seo, S. S., Louros, S. R., & Osterweil, E. K. (2020) · eNeuro · doi:10.1523/eneuro.0162-20.2020 · Cited by 6 · see citing works
Self-citationThe Use of Peptides in the Treatment of Fragile X Syndrome: Challenges and Opportunities
Romagnoli, A., & Marino, D. D. (2021) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.754485 · Cited by 7 · see citing works
Morin‐Parent, F., Champigny, C. M., Cote, S. R., Mohamad, T., Hasani, S. A., Çaku, A., Corbin, F., & Lepage, J. (2024) · Autism Research · doi:10.1002/aur.3222 · Cited by 5 · see citing works
McKinney, W. S., Schmitt, L., Stefano, L. A. D., Ethridge, L. E., Norris, J. E., Horn, P. S., Dauterman, S., Rosselot, H., Pedapati, E. V., Reisinger, D. L., et al. (2024) · Journal of Child and Adolescent Psychopharmacology · doi:10.1089/cap.2024.0103 · Cited by 5 · see citing works
Pharmacological Treatments for Fragile X Syndrome Based on Synaptic Dysfunction
Telias, M. (2019) · Current Pharmaceutical Design · doi:10.2174/1381612825666191102165206 · Cited by 4 · see citing works
Is cholesterol both the lock and key to abnormal transmembrane signals in Autism Spectrum Disorder?
Lingwood, C. A. (2024) · Lipids in Health and Disease · doi:10.1186/s12944-024-02075-3 · Cited by 3 · see citing works
Möhrle, D., Ma, D., Xue, W., Yan, J., & Cheng, N. (2025) · Autism Research · doi:10.1002/aur.70152 · Cited by 3 · see citing works
A scoping review on natural cholesterol lowering supplements sold in South African pharmacies
Lee, H. B., Khan, R., Vally, M., & Orchard, A. (2024) · Health SA Gesondheid · doi:10.4102/hsag.v29i0.2299 · Cited by 2 · see citing works
Mechanisms of proteostasis in neuronal development and plasticity
Maio, B., Aria, F., Louros, S. R., & Osterweil, E. K. (2025) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2025.103143 · Cited by 1 · see citing works
Self-citationRosenhouse‐Dantsker, A., Slayden, A. V., & Bukiya, A. N. (2022) · Elsevier eBooks · doi:10.1016/b978-0-323-85857-1.00002-x · Cited by 1 · see citing works
Fenton, T. A., Petkova, S. P., Adhikari, A., & Silverman, J. L. (2025) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-025-09616-6
Maio, B., Singh, A., Hector, R., Gadalla, K. K. E., Selfridge, J., Aria, F., Louros, S. R., Cobb, S., & Osterweil, E. K. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.06.12.731901
Self-citationHuang, W., Jiang, L., Zhu, Y., Duan, R., Xue, J., & Xia, Q. (2026) · Molecular Neurobiology · doi:10.1007/s12035-026-06115-0
- Authors
- Muscas, M., Louros, S. R., & Osterweil, E. K.
- Affiliations
- Simons Initiative for the Developing Brain (GB)
- Venue
- eneuro
- Publisher
- Society for Neuroscience
- Published
- 2019-05
- Volume
- 6
- Issue
- 3
- Pages
- ENEURO.0097-19.2019
- ISSN
- 2373-2822 (ISSN-L)
- PMC
- PMC6565377
- OpenAlex
- W2951046587
- Cited by
- 30
- References
- 61
- Citations by year
- 2026: 2 · 2025: 3 · 2024: 5 · 2023: 2 · 2022: 5 · 2021: 4 · 2020: 7 · 2019: 2
- FWCI
- 2.71
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- eNeuro (publishedVersion, cc-by)
- bioRxiv (Cold Spring Harbor Laboratory) (acceptedVersion)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
- Funders
- Wellcome Trust (Sir Henry Dale fellowship 104116/Z/14/Z)
- Medical Research Council (MR/M006336/1)
- Simons Initiative for the Developing Brain
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Keywords
- lovastatin, simvastatin, fragile X syndrome, Fmr1 knockout mice, audiogenic seizures, statins, autism spectrum disorder, intellectual disability, drug comparison
- MeSH terms
- Acoustic Stimulation, Animals, Anticholesteremic Agents, Disease Models, Animal, Fragile X Syndrome, Hippocampus, Lovastatin, Male, Mice, Inbred C57BL, Mice, Transgenic, Phenotype, Protein Biosynthesis, Simvastatin, Epilepsy, Reflex, MAP Kinase Signaling System
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
