Morphological and functional alterations in the substantia nigra pars compacta of the Mecp2-null mouse
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Rett syndrome (RTT) is a severe neurological disorder caused by mutations in the MECP2 gene, in which older patients often develop parkinsonian features. Although Mecp2 has been shown to modulate the catecholaminergic metabolism of the RTT mouse model, little is known about the central dopaminergic neurons. Here we found that the progression of the motor dysfunction in the Mecp2-deficient mouse becomes more severe between 4 and 9 weeks of age. We then studied the phenotype of the dopaminergic neurons of the substantia nigra pars compacta (SNpc). We found a major reduction in the number of tyrosine hydroxylase (Th)-expressing neurons, as well as a reduction in their soma size, by 5 weeks of age. We showed that this deficit is not due to apoptosis and that the remaining neurons express a mature dopaminergic phenotype. A reduction in the Th-staining intensity was also found in the caudate-putamen (CPu), the main dopaminergic target for SNpc. We found that the amount of activated-Th (pSer40-Th) is slightly reduced at 5 weeks of age in the Mecp2-deficient mouse, but that this amount is affected more importantly by 9 weeks of age. Neurochemical measurements revealed a significant reduction of dopamine content at 5 and 9 weeks of age in the CPu whereas SNpc contents were preserved. Finally, we found that chronic L-Dopa treatment improved the motor deficits previously identified. Altogether, our findings demonstrate that Mecp2-deficiency induces nigrostriatal deficits, and they offer a new perspective to better understand the origin of motor dysfunction in RTT.
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In an engineered MeCP2-deficient mouse model, a postnatal reduction of DA and its metabolite homovanillic acid was seen in the caudate putamen.
“In an engineered MeCP2-deficient mouse model, a postnatal reduction of DA and its metabolite homovanillic acid was seen in the caudate putamen [701,702]”
From Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings (2012) · cites this paper as [702]
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- Authors
- Panayotis, N., Pratte, M., Borges-Correia, A., Ghata, A., Villard, L., & Roux, J.-C.
- Affiliations
- Inserm (FR)
- Corresponding author
- Panayotis, Nicolas
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 2011-02
- Volume
- 41
- Issue
- 2
- Pages
- 385-397
- ISSN
- 0969-9961 (ISSN-L)
- OpenAlex
- W2018107367
- Cited by
- 54
- References
- 58
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 2 · 2023: 1 · 2022: 2 · 2021: 7 · 2019: 5 · 2018: 4 · 2017: 3 · 2016: 3 · 2015: 4 · 2014: 5 · 2013: 7 · 2012: 3
- FWCI
- 2.70
- Type
- journal article
- Language
- en
- Open access
- green
- doaj.org
- Licence
- Locations
- Neurobiology of Disease (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- Institut National de la Santé et de la Recherche Médicale
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Nuclear Receptors and Signaling
- Keywords
- Rett syndrome, substantia nigra pars compacta, dopaminergic neurons, tyrosine hydroxylase, nigrostriatal pathway, caudate-putamen, dopamine content, motor impairment, parkinsonian features, L-dopa treatment, neuronal soma size
- MeSH terms
- Animals, Disease Models, Animal, Dopamine, Male, Mice, Inbred C57BL, Mice, Transgenic, Motor Neurons, Phenotype, Substantia Nigra, Rett Syndrome, Mice, Knockout, Disease Progression, Mice, Methyl-CpG-Binding Protein 2
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