期刊
BIOESSAYS
卷 31, 期 5, 页码 561-569出版社
WILEY
DOI: 10.1002/bies.200800220
关键词
HDAC inhibitor therapeutics; MeCP2-chromatin; PEST domains; proteolytic turnover; Rett syndrome
资金
- Michael Smith Foundation for Health Research (MSFHR)
- Canadian Institutes of Health Research (CIHR)
- Natural Science and Engineering Research Council of Canada (NSERC)
- Ontario Rett Syndrome Association (ORSA)
- Stroke Foundation of Canada
Mutations in the methyl-CpG-binding protein 2 (MeCP2) cause Rett syndrome, a severe neurodevelopmental disease associated with ataxia and other post-natal symptoms similar to autism. Much research interest has focussed on the implications of MeCP2 in disease and neuron physiology. However, little or no attention has been paid to how MeCP2 turnover is regulated. The post-translational control of MeCP2 is of critical importance, especially as subtle increases or decreases in MeCP2 amounts can affect neuron morphology and function. The latter point is of particular importance for gene therapeutic approaches in which exogenous wild-type MeCP2 is being introduced into diseased neurons. Further to this, we propose two hypotheses. The first hypothesis discusses the polyubiquitin-mediated post-translational regulation of MeCP2 through its two PEST domains. The second hypothesis explores the use of histone deacetylase inhibitors to modulate the amounts of MeCP2 expressed in conjunction with the aforementioned therapeutic approaches.
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