Preclinical research in Rett syndrome: setting the foundation for translational success
Open accessAbstract
In September of 2011, the National Institute of Neurological Disorders and Stroke (NINDS), the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), the International Rett Syndrome Foundation (IRSF) and the Rett Syndrome Research Trust (RSRT) convened a workshop involving a broad cross-section of basic scientists, clinicians and representatives from the National Institutes of Health (NIH), the US Food and Drug Administration (FDA), the pharmaceutical industry and private foundations to assess the state of the art in animal studies of Rett syndrome (RTT). The aim of the workshop was to identify crucial knowledge gaps and to suggest scientific priorities and best practices for the use of animal models in preclinical evaluation of potential new RTT therapeutics. This review summarizes outcomes from the workshop and extensive follow-up discussions among participants, and includes: (1) a comprehensive summary of the physiological and behavioral phenotypes of RTT mouse models to date, and areas in which further phenotypic analyses are required to enhance the utility of these models for translational studies; (2) discussion of the impact of genetic differences among mouse models, and methodological differences among laboratories, on the expression and analysis, respectively, of phenotypic traits; and (3) definitions of the standards that the community of RTT researchers can implement for rigorous preclinical study design and transparent reporting to ensure that decisions to initiate costly clinical trials are grounded in reliable preclinical data.
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In wild-type primary visual cortex, the time course of activation in layers 2/3, 4, 5, and 6 shows a significant decrease in amplitude at postnatal day 30 compared with postnatal day 15.
“Time course of activation in WT L2/3 (G), L4 (H), L5 (I) and L6 (J) show significant decrease in amplitude at P30 (black), compared with P15 (76).”
From MeCP2 regulates the timing of critical period plasticity that shapes functional connectivity in primary visual cortex (2015) · cites this paper as [76]
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7,8-dihydroxyflavone exhibits therapeutic efficacy in a mouse model of Rett syndrome
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Electrocardiographic findings in Rett syndrome: An explanation for sudden death?
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Postnatal dietary choline supplementation alters behavior in a mouse model of Rett syndrome
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The fallacy of behavioral phenotyping without standardisation
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Diagnostic criteria for the Zappella variant of Rett syndrome (the preserved speech variant)
Renieri, A., Mari, F., Mencarelli, M. A., Scala, E., Ariani, F., Longo, I., Meloni, I., Cevenini, G., Pini, G., Hayek, J., et al. (2008) · Brain and Development · doi:10.1016/j.braindev.2008.04.007 · Cited by 100 · see citing works
Early postnatal behavioral changes in the Mecp2‐308 truncation mouse model of Rett syndrome
Filippis, B. D., Ricceri, L., & Giovanni Laviola, A. (2009) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2009.00551.x · Cited by 99 · see citing works
An altered neonatal behavioral phenotype in Mecp2 mutant mice
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Self-citationMeCP2 and Rett syndrome: reversibility and potential avenues for therapy
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Linking MECP2 and pain sensitivity: The example of Rett syndrome
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Evidence for abnormal early development in a mouse model of Rett syndrome
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Postnatal inactivation reveals enhanced requirement for MeCP2 at distinct age windows
Cheval, H., Guy, J., Merusi, C., Sousa, D. D., Selfridge, J., & Bird, A. P. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds208 · Cited by 95 · see citing works
Self-citationExtrapyramidal involvement in Rett's syndrome
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Self-citationPolysomnographic characteristics of patients with Rett syndrome
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Oral treatment with desipramine improves breathing and life span in Rett syndrome mouse model
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MeCP2 Is Critical within HoxB1-Derived Tissues of Mice for Normal Lifespan
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Self-citationAlterations of cortical and hippocampal EEG activity in MeCP2-deficient mice
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Self-citationFeatures of Autism in Rett Syndrome and Severe Mental Retardation
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Self-citationAbnormal Expression of Microtubule-Associated Protein 2 (MAP-2) in Neocortex in Rett Syndrome
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Cognitive performance in Rett syndrome girls: a pilot study using eyetracking technology
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Early breathing defects after moderate hypoxia or hypercapnia in a mouse model of Rett syndrome
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Profiling Scoliosis in Rett Syndrome
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Self-citationUbe3a mRNA and protein expression are not decreased in Mecp2 mutant mice
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Self-citationLoss of Mecp2 in Substantia Nigra Dopamine Neurons Compromises the Nigrostriatal Pathway
Gantz, S. C., Ford, C. P., Neve, K. A., & Williams, J. T. (2011) · The Journal of Neuroscience · doi:10.1523/jneurosci.0684-11.2011 · Cited by 62 · see citing works
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Separate Respiratory Phenotypes in Methyl-CpG-Binding Protein 2 (Mecp2) Deficient Mice
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Synaptic determinants of Rett syndrome
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Self-citationQuestioning standardization in science
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EEG and respiration in Rett syndrome
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Comparative psychology and the grand challenge of drug discovery in psychiatry and neurodegeneration
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Behaviour problems in adult women with Rett syndrome
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Mecp2 Truncation in Male Mice Promotes Affiliative Social Behavior
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Face to Face with Rett Syndrome
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Rett syndrome: a neurological disorder with metabolic components
Kyle, S. M., Vashi, N., & Justice, M. J. (2018) · Open Biology · doi:10.1098/rsob.170216 · Cited by 213 · see citing works
Self-citationMeCP2 co-ordinates liver lipid metabolism with the NCoR1/HDAC3 corepressor complex
Kyle, S. M., Saha, P. K., Brown, H. M., Chan, L. C., & Justice, M. J. (2016) · Human Molecular Genetics · doi:10.1093/hmg/ddw156 · Cited by 84 · see citing works
Self-citationRecent advances in gene therapy for neurodevelopmental disorders with epilepsy
Turner, T. J., Zourray, C., Schorge, S., & Lignani, G. (2021) · Journal of Neurochemistry · doi:10.1111/jnc.15168 · Cited by 71 · see citing works
Kron, M., Lang, M., Adams, I. T., Sceniak, M., Longo, F., & Katz, D. M. (2014) · Disease Models & Mechanisms · doi:10.1242/dmm.016030 · Cited by 59 · see citing works
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Lovastatin fails to improve motor performance and survival in methyl-CpG-binding protein2-null mice
Villani, C., Sacchetti, G., Bagnati, R., Passoni, A., Fusco, F., Carli, M., & Invernizzi, R. W. (2016) · eLife · doi:10.7554/elife.22409 · Cited by 20 · see citing works
- Authors
- Katz, D. M., Berger-Sweeney, J. E., Eubanks, J. H., Justice, M. J., Neul, J. L., Pozzo-Miller, L., Blue, M. E., Christian, D., Crawley, J. N., Giustetto, M., Guy, J., Howell, C. J., Kron, M., Nelson, S. B., Samaco, R. C., Schaevitz, L. R., St. Hillaire-Clarke, C., Young, J. L., Zoghbi, H. Y., & Mamounas, L. A.
- Affiliations
- Case Western Reserve University (US)
- Tufts University (US)
- Toronto Western Research Institute, 399 Bathurst Street, Mac 11-408, Toronto, ON M5T 2S8, Canada
- Baylor College of Medicine (US)
- Department of Neurobiology, The University of Alabama at Birmingham, 1825 University Boulevard, SHEL-1002, Birmingham, AL 35294, USA
- Kennedy Krieger Institute (US)
- University of California, Davis (US)
- University of Turin (IT)
- University of Edinburgh (GB)
- Brandeis University (US)
- National Institute of Neurological Disorders and Stroke, National Institutes of Health, 6001 Executive Boulevard, Bethesda, MD 20892, USA
- University of Miami (US)
- Howard Hughes Medical Institute (US)
- National Institutes of Health (US)
- Corresponding author
- Katz, David M.
- Zoghbi, Huda Y.
- Mamounas, Laura A.
- ORCID
- Venue
- Disease Models & Mechanisms
- Publisher
- The Company of Biologists
- Published
- 2012-11-01
- Volume
- 5
- Issue
- 6
- Pages
- 733-745
- ISSN
- 1754-8403 (ISSN-L)
- 1754-8411
- PMC
- PMC3484856
- OpenAlex
- W2104054545
- Cited by
- 206
- References
- 143
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 11 · 2023: 9 · 2022: 13 · 2021: 16 · 2020: 22 · 2019: 10 · 2018: 17 · 2017: 22 · 2016: 17 · 2015: 22 · 2014: 23 · 2013: 15 · 2012: 1
- FWCI
- 2.41
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by-nc-sa
- Locations
- Disease Models & Mechanisms (publishedVersion, cc-by-nc-sa)
- PubMed Central (publishedVersion, cc-by-nc-sa)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion, cc-by-nc-sa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Funders
- Howard Hughes Medical Institute
- International Rett Syndrome Foundation
- Rett Syndrome Research Trust
- National Institutes of Health
- Canadian Institutes of Health Research (106481_1)
- National Human Genome Research Institute (U54HG006348)
- National Institute of Neurological Disorders and Stroke (R01NS065027, NS‐065027, T32NS043124, R01NS057398, R56NS057398, NS-057398)
- National Institute of Child Health and Human Development
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (R01HD062553)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Health Sciences › Veterinary › Small Animals › Animal testing and alternatives
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Keywords
- Rett syndrome, animal models, mouse model, preclinical research, translational research, phenotyping, preclinical study design, reporting transparency, treatment evaluation, research priorities
- MeSH terms
- Animals, Congresses as Topic, Disease Models, Animal, Humans, Rett Syndrome, Guidelines as Topic, Translational Research, Biomedical, Research Report
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.
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