Systemic radical scavenger treatment of a mouse model of Rett syndrome: merits and limitations of the vitamin E derivative Trolox
Open accessAbstract
Rett syndrome (RTT) is a severe neurodevelopmental disorder typically arising from spontaneous mutations in the X-chromosomal methyl-CpG binding protein 2 (MECP2) gene. The almost exclusively female Rett patients show an apparently normal development during their first 6-18 months of life. Subsequently, cognitive- and motor-impairment, hand stereotypies, loss of learned skills, epilepsy, and irregular breathing manifest. Early mitochondrial impairment and oxidative challenge are considered to facilitate disease progression. Along this line, we recently confirmed in vitro that acute treatment with the vitamin E-derivative Trolox dampens neuronal hyperexcitability, reinstates synaptic plasticity, ameliorates cellular redox balance, and improves hypoxia tolerance in male MeCP2-deficient (Mecp2-/y) mouse hippocampus. Pursuing these promising findings, we performed a preclinical study to define the merit of systemic Trolox administration. Blinded, placebo-controlled in vivo treatment of male mice started at postnatal day 10-11 and continued for ~40 days. Compounds (vehicle only, 10 mg/kg or 40 mg/kg Trolox) were injected intraperitoneally every 48h. Detailed phenotyping revealed that in Mecp2-/y mice, blood glucose levels, lipid peroxidation, synaptic short-term plasticity, hypoxia tolerance, and certain forms of environmental exploration were improved by Trolox. Yet, body weight and size, motor function, and the rate and regularity of breathing did not improve. In conclusion, in vivo Trolox treatment partially ameliorated a subset of symptoms of the complex Rett phenotype, thereby confirming a partial merit of the vitamin E-derivative based pharmacotherapy. Yet, it also became evident that frequent animal handling and the route of drug administration are critical issues to be optimized in future trials.
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Trolox, a vitamin E derivative, normalized blood glucose levels, reduced oxidative stress and improved exploratory behaviour in Mecp2 mice.
“Similarly, Trolox, a vitamin E derivative, normalized blood glucose levels, reduced oxidative stress and improved exploratory behaviour in Mecp2 mice [208].”
From Rett syndrome: a neurological disorder with metabolic components (2018) · cites this paper as [208]
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Vashi, N., & Justice, M. J. (2019) · Mammalian Genome · doi:10.1007/s00335-019-09793-5 · Cited by 111 · see citing works
Drug Studies on Rett Syndrome: From Bench to Bedside
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Bebensee, D. F., Can, K., & Müller, M. (2017) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2017/3064016 · Cited by 48 · see citing works
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4-hydroxynonenal protein adducts: Key mediator in Rett syndrome oxinflammation
Valacchi, G., Pecorelli, A., Cervellati, C., & Hayek, J. (2017) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2016.12.045 · Cited by 37 · see citing works
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Research models of neurodevelopmental disorders: The right model in the right place
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Transcriptome level analysis in Rett syndrome using human samples from different tissues
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MECP2 Duplication Syndrome: Evidence of Enhanced Oxidative Stress. A Comparison with Rett Syndrome
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Persistent Expression of Serotonin Receptor 5b Alters Breathing Behavior in Male MeCP2 Knockout Mice
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Lung Defects Contribute to Respiratory Symptoms in a Mecp2-mutant Mouse Model of Rett Syndrome
Vashi, N. (2021) · TSpace (University of Toronto)
Understanding Rett Syndrome: By Means of Genetic Mutations and Evolutionary Classification
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- Authors
- Janc, O. A., Hüser, M. A., Dietrich, K., Kempkes, B., Menzfeld, C., Hülsmann, S., & Müller, M.
- Affiliations
- Universitätsmedizin Göttingen (DE)
- Corresponding author
- Müller, Michael
- Venue
- Frontiers in Cellular Neuroscience
- Publisher
- Frontiers Media SA
- Published
- 2016-11-15
- Volume
- 10
- Pages
- 266-266
- ISSN
- 1662-5102 (ISSN-L)
- PMC
- PMC5109403
- OpenAlex
- W2555189562
- Cited by
- 35
- References
- 87
- Citations by year
- 2024: 1 · 2023: 3 · 2022: 4 · 2021: 11 · 2020: 1 · 2019: 5 · 2018: 6 · 2017: 3 · 2016: 1
- FWCI
- 4.04
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Frontiers in Cellular Neuroscience (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- GoeScholar The Publication Server of the Georg-August-Universität Göttingen (Georg-August-Universität Göttingen) (submittedVersion, cc-by)
- GRO (submittedVersion, cc-by)
- Funders
- Deutsche Forschungsgemeinschaft (CNMPB, regular research grant 2817)
- International Rett Syndrome Foundation
- 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 › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- Rett syndrome, Trolox, vitamin E derivatives, MeCP2, oxidative stress, mitochondrial dysfunction, synaptic plasticity, hypoxia tolerance, lipid peroxidation, neuronal hyperexcitability, redox homeostasis, pharmacotherapy, route of administration, animal handling
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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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