Evaluation of Rett Syndrome Symptom Improvement by Metabolic Modulators inMecp2-Mutant Mice

摘要: Mouse models recapitulate many symptoms of Rett Syndrome, an X-linked disorder caused by mutations in methyl-CpG-binding protein 2 (MECP2). The study Mecp2-null male mice has provided insight into pathogenesis the disorder-most recently, dysregulation cholesterol and lipid metabolism. Perisymptomatic treatment with statin drugs successfully mitigates effects this metabolic syndrome, increases longevity, improves motor function. Described here is a drug screening protocol timeline for symptom evaluation Mecp2-mutant mice. Specifically, are treated twice weekly compound interest alongside subjective health assessments, bi-weekly body composition measurements, blood chemistries. Throughout treatment, behavioral phenotyping tests carried out at specific time points. This highly adaptable to other neurological diseases; however, completion depends on mutant model under study. highlights use techniques described several different Current Protocols Biology articles carry testing preclinical model. Curr. Protoc. Biol. 3:187-204 © 2013 John Wiley & Sons, Inc.