| 项目编号 |
PRJCA000236 |
| 项目标题 |
Loss of Asxl1 Alters Self-Renewal and Cell Fate of Bone Marrow Stromal Cell |
| 涉及领域 |
Model organism |
| 数据类型 |
Transcriptome or Gene expression
|
| 物种名称 |
Mus musculus
|
| 描述信息 |
De novo ASXL1 mutations are found in patients with Bohring-Opitz syndrome, a disease with severe developmental defects and early childhood fatality. The underlying pathologic mechanisms remain largely unknown. Using Asxl1-targeted murine models,we found that Asxl1 global loss or conditional deletion in osteoblasts and their progenitors in mice leads to significant bone loss and markedly decreased numbers of marrow mesenchymal stem/progenitor cells (MSPCs) compared with wild-type (WT) littermates. Asxl1-/- MSPCs displayed impaired self-renewal and skewed differentiation-away from osteoblasts and favoring adipocytes. RNA-seq analysis reveals the altered expression of genes involved in cell proliferation, skeletal development and morphogenesis. Furthermore, gene set enrichment analysis showed a decreased gene expression of stem cell self-renewal signature,suggesting the role of Asxl1 in regulating the stemness of MSPCs. Importantly, introducing Asxl1 normalized NANOG and OCT4 expression and restored the self-renewal capacity of Asxl1-/- MSPCs. Our study unveils a pivotal role of ASXL1 in maintenance of MSPC functions and skeletal development. |
| 样品范围 |
Monoisolate |
| 发布日期 |
2016-05-19 |
| 出版信息 |
| PubMed ID |
文章标题 |
杂志名称 |
Doi |
发表年份 |
| 27237378
|
Loss of Asxl1 Alters Self-Renewal and Cell Fate of Bone Marrow Stromal Cell, Leading to Bohring-Opitz-like Syndrome in Mice.
|
Stem Cell Reports
|
10.1016/j.stemcr.2016.04.013
|
2016
|
|
| 实验材料提供者 |
Feng-Chun Yang |
| 项目资金来源 |
| 机构 |
项目类型 |
授权项目ID |
授权项目名称 |
| National Institutes of Health
|
|
CA172408
|
CA172408
|
| National Institutes of Health
|
|
CA185751
|
CA185751
|
| National Institutes of Health
|
|
HL112294
|
HL112294
|
| National Natural Science Foundation of China (NSFC)
|
|
81270612
|
81270612
|
| Chinese Academy of Sciences (CAS)
|
|
153F11KYSB20150013
|
153F11KYSB20150013
|
|
| 本项目在其他数据库系统中的项目序列号 |
| 项目序列号 |
数据库名称 |
| GSE75787
|
NCBI
|
|
| 项目相关网站链接 |
|
| 提交者 |
Fuhong
He (hefh@big.ac.cn)
|
| 提交单位 |
Beijing Institute of Genomics, Chinese Academy of Sciences |
| 提交日期 |
2016-05-19 |