| RRC ID |
42900
|
| 著者 |
Cui CH, Uyama T, Miyado K, Terai M, Kyo S, Kiyono T, Umezawa A.
|
| タイトル |
Menstrual blood-derived cells confer human dystrophin expression in the murine model of Duchenne muscular dystrophy via cell fusion and myogenic transdifferentiation.
|
| ジャーナル |
Mol Biol Cell
|
| Abstract |
Duchenne muscular dystrophy (DMD), the most common lethal genetic disorder in children, is an X-linked recessive muscle disease characterized by the absence of dystrophin at the sarcolemma of muscle fibers. We examined a putative endometrial progenitor obtained from endometrial tissue samples to determine whether these cells repair muscular degeneration in a murine mdx model of DMD. Implanted cells conferred human dystrophin in degenerated muscle of immunodeficient mdx mice. We then examined menstrual blood-derived cells to determine whether primarily cultured nontransformed cells also repair dystrophied muscle. In vivo transfer of menstrual blood-derived cells into dystrophic muscles of immunodeficient mdx mice restored sarcolemmal expression of dystrophin. Labeling of implanted cells with enhanced green fluorescent protein and differential staining of human and murine nuclei suggest that human dystrophin expression is due to cell fusion between host myocytes and implanted cells. In vitro analysis revealed that endometrial progenitor cells and menstrual blood-derived cells can efficiently transdifferentiate into myoblasts/myocytes, fuse to C2C12 murine myoblasts by in vitro coculturing, and start to express dystrophin after fusion. These results demonstrate that the endometrial progenitor cells and menstrual blood-derived cells can transfer dystrophin into dystrophied myocytes through cell fusion and transdifferentiation in vitro and in vivo.
|
| 巻・号 |
18(5)
|
| ページ |
1586-94
|
| 公開日 |
2007-5-1
|
| DOI |
10.1091/mbc.e06-09-0872
|
| PII |
E06-09-0872
|
| PMID |
17314403
|
| PMC |
PMC1855042
|
| MeSH |
Animals
Cell Differentiation
Cell Fusion
Cell Line
Dystrophin / genetics*
Endometrium / cytology
Female
Gene Transfer Techniques
Humans
In Vitro Techniques
Menstruation / blood*
Mice
Mice, Inbred NOD
Mice, Inbred mdx
Mice, Knockout
Mice, SCID
Muscle Development
Muscular Dystrophy, Animal / genetics
Muscular Dystrophy, Animal / pathology
Muscular Dystrophy, Animal / therapy*
Muscular Dystrophy, Duchenne / genetics
Muscular Dystrophy, Duchenne / therapy
|
| IF |
3.791
|
| 引用数 |
118
|
|
WOS 分野
|
CELL BIOLOGY
|
| リソース情報 |
| ヒト・動物細胞 |
C2C12(RCB0987) |
