Lee Yun-Sil, Talbot C Conover, Lee Se-Jin
Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, 725 North Wolfe Street, PCTB 803, Baltimore, MD 21205, USA.
Institute for Basic Biomedical Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Genom Data. 2015 Oct 24;6:253-7. doi: 10.1016/j.gdata.2015.10.010. eCollection 2015 Dec.
Because myostatin normally limits skeletal muscle growth, there are extensive efforts to develop myostatin inhibitors for clinical use. One potential concern is that in muscle degenerative diseases, inducing hypertrophy may increase stress on dystrophic fibers. Our study shows that blocking this pathway in dysferlin deficient mice results in early improvement in histopathology but ultimately accelerates muscle degeneration. Hence, benefits of this approach should be weighed against these potential detrimental effects. Here, we present detailed experimental methods and analysis for the gene expression profiling described in our recently published study in Human Molecular Genetics (Lee et al., 2015). Our data sets have been deposited in the Gene Expression Omnibus (GEO) database (GSE62945) and are available at http://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE62945. Our data provide a resource for exploring molecular mechanisms that are related to hypertrophy-induced, accelerated muscular degeneration in dysferlinopathy.
由于肌生成抑制素通常会限制骨骼肌生长,因此人们为开发用于临床的肌生成抑制素抑制剂付出了巨大努力。一个潜在的担忧是,在肌肉退行性疾病中,诱导肥大可能会增加对营养不良纤维的压力。我们的研究表明,在dysferlin缺陷小鼠中阻断该途径会导致组织病理学早期改善,但最终会加速肌肉退化。因此,应权衡这种方法的益处与这些潜在的有害影响。在此,我们展示了我们最近发表在《人类分子遗传学》(Lee等人,2015年)中的研究中所描述的基因表达谱分析的详细实验方法和分析。我们的数据集已存入基因表达综合数据库(GEO)(GSE62945),可在http://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE62945获取。我们的数据为探索与dysferlin病中肥大诱导的加速肌肉退化相关的分子机制提供了资源。